SUBJECT SEMINAR MOTILITY DISORDERS OF ESOPHAGUS Chairperson- Dr. Vijay Kamat Associate Professor Department of General Surgery KIMS Speaker- Dr Mohammad Amir PG Student Department of General Surgery Date- 7 th June 2016 Time- 2:30 PM Venue- Surgery Seminar Hall
Associate ProfessorDepartment of General SurgeryKIMS
Speaker- Dr Mohammad AmirPG StudentDepartment of General Surgery
Date- 7th June 2016Time- 230 PMVenue- Surgery Seminar Hall
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
The tissues forming the thoracic oesophageal wall from lumen outwards are the mucosa (consisting of epithelium lamina propria and muscularis mucosae) submucosa muscularis externa and adventitia
Epithelium is a non-keratinized stratified squamous epithelium
Areas of weakness
ENTERIC NERVOUS SYSTEM
Two Plexus
MYENTERIC PLEXUS
Auerbachrsquos plexusOuterlongitudinal and circular layer
MYENTERIC PLEXUS Linear chain of many interconnecting neurons that extends the entire
length of GIT Between the longitudinal and circular layers of intestinal smooth muscle Muscle activity along the length of the gut Stimulation
1 Increased tonic contraction or ldquotonerdquo of the gut wall2 Increased intensity of the rhythmical contractions3 Increased rate of the rhythm of contraction4 Increased velocity of conduction of excitatory waves along the gut
wall causing more rapid movement of the gut peristaltic waves
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
The tissues forming the thoracic oesophageal wall from lumen outwards are the mucosa (consisting of epithelium lamina propria and muscularis mucosae) submucosa muscularis externa and adventitia
Epithelium is a non-keratinized stratified squamous epithelium
Areas of weakness
ENTERIC NERVOUS SYSTEM
Two Plexus
MYENTERIC PLEXUS
Auerbachrsquos plexusOuterlongitudinal and circular layer
MYENTERIC PLEXUS Linear chain of many interconnecting neurons that extends the entire
length of GIT Between the longitudinal and circular layers of intestinal smooth muscle Muscle activity along the length of the gut Stimulation
1 Increased tonic contraction or ldquotonerdquo of the gut wall2 Increased intensity of the rhythmical contractions3 Increased rate of the rhythm of contraction4 Increased velocity of conduction of excitatory waves along the gut
wall causing more rapid movement of the gut peristaltic waves
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
The tissues forming the thoracic oesophageal wall from lumen outwards are the mucosa (consisting of epithelium lamina propria and muscularis mucosae) submucosa muscularis externa and adventitia
Epithelium is a non-keratinized stratified squamous epithelium
Areas of weakness
ENTERIC NERVOUS SYSTEM
Two Plexus
MYENTERIC PLEXUS
Auerbachrsquos plexusOuterlongitudinal and circular layer
MYENTERIC PLEXUS Linear chain of many interconnecting neurons that extends the entire
length of GIT Between the longitudinal and circular layers of intestinal smooth muscle Muscle activity along the length of the gut Stimulation
1 Increased tonic contraction or ldquotonerdquo of the gut wall2 Increased intensity of the rhythmical contractions3 Increased rate of the rhythm of contraction4 Increased velocity of conduction of excitatory waves along the gut
wall causing more rapid movement of the gut peristaltic waves
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
MICROSTRUCTURE
The tissues forming the thoracic oesophageal wall from lumen outwards are the mucosa (consisting of epithelium lamina propria and muscularis mucosae) submucosa muscularis externa and adventitia
Epithelium is a non-keratinized stratified squamous epithelium
Areas of weakness
ENTERIC NERVOUS SYSTEM
Two Plexus
MYENTERIC PLEXUS
Auerbachrsquos plexusOuterlongitudinal and circular layer
MYENTERIC PLEXUS Linear chain of many interconnecting neurons that extends the entire
length of GIT Between the longitudinal and circular layers of intestinal smooth muscle Muscle activity along the length of the gut Stimulation
1 Increased tonic contraction or ldquotonerdquo of the gut wall2 Increased intensity of the rhythmical contractions3 Increased rate of the rhythm of contraction4 Increased velocity of conduction of excitatory waves along the gut
wall causing more rapid movement of the gut peristaltic waves
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Areas of weakness
ENTERIC NERVOUS SYSTEM
Two Plexus
MYENTERIC PLEXUS
Auerbachrsquos plexusOuterlongitudinal and circular layer
MYENTERIC PLEXUS Linear chain of many interconnecting neurons that extends the entire
length of GIT Between the longitudinal and circular layers of intestinal smooth muscle Muscle activity along the length of the gut Stimulation
1 Increased tonic contraction or ldquotonerdquo of the gut wall2 Increased intensity of the rhythmical contractions3 Increased rate of the rhythm of contraction4 Increased velocity of conduction of excitatory waves along the gut
wall causing more rapid movement of the gut peristaltic waves
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
ENTERIC NERVOUS SYSTEM
Two Plexus
MYENTERIC PLEXUS
Auerbachrsquos plexusOuterlongitudinal and circular layer
MYENTERIC PLEXUS Linear chain of many interconnecting neurons that extends the entire
length of GIT Between the longitudinal and circular layers of intestinal smooth muscle Muscle activity along the length of the gut Stimulation
1 Increased tonic contraction or ldquotonerdquo of the gut wall2 Increased intensity of the rhythmical contractions3 Increased rate of the rhythm of contraction4 Increased velocity of conduction of excitatory waves along the gut
wall causing more rapid movement of the gut peristaltic waves
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
MYENTERIC PLEXUS Linear chain of many interconnecting neurons that extends the entire
length of GIT Between the longitudinal and circular layers of intestinal smooth muscle Muscle activity along the length of the gut Stimulation
1 Increased tonic contraction or ldquotonerdquo of the gut wall2 Increased intensity of the rhythmical contractions3 Increased rate of the rhythm of contraction4 Increased velocity of conduction of excitatory waves along the gut
wall causing more rapid movement of the gut peristaltic waves
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Function of the Myenteric Plexus in Peristalsis
Effectual peristalsis requires an active myenteric plexus
Weak or absent in any portion of the gastrointestinal tract that has congenital absence of the myenteric plexus
Depressed or completely blocked in the entire gut when a person is treated with atropine to paralyze the cholinergic nerve endings of the myenteric plexus
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
NEURAL CONTROL OF LES
Release of acetylcholine from vagal endings causes the intrinsic sphincter to contract
Release of NO and VIP from interneurons innervated by other vagal fibers causes it to relax
Contraction of the crural portion of the diaphragm which is innervated by the phrenic nerves is coordinated with respiration and contractions of chest and abdominal muscles
Thus the intrinsic and extrinsic sphincters operate together to permit orderly flow of food into the stomach and to prevent reflux
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
MOTILITY DISORDERS OF ESOPHAGUS
Disorders of the esophageal phase of swallowing due to abnormalities in the propulsive pump action of the esophageal body or the relaxation of the LES
Classified based on etiology or anatomical location
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
ACHALASIA
Achalasia- Greek origin (failure to relax) First described by Willis in 1674 Affecting 6 in 100000 individuals Second most common functional disorder of the esophagus after
GERD Arthur Hurst- achalasia of the cardia
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
ACOG DEFINITION OF ACHALASIAAchalasia is a primary esophageal motor disorder of unknown etiology characterized- manometrically by insufficient relaxation of the lower esophageal
sphincter (LES) and loss of esophageal peristalsis radiographically by aperistalsis esophageal dilation with minimal
LES opening ldquo bird-beak rdquo appearance poor emptying of barium endoscopically by dilated esophagus with retained saliva liquid and
undigested food particles in the absence of mucosal stricturing or tumor
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Complex motor abnormality of the esophageal body and LES with loss of the ganglion cells in the myenteric (Auerbachrsquos) plexus
The exact cause is unknown with data suggesting hereditary degenerative autoimmune and infectious features as possible causes
Currently achalasia is an idiopathic condition that is associated with T-lymphocyte eosinophil and mast cell infiltration in the sophageal myenteric (Auerbach) plexus with myenteric neural fibrosis hypertrophy of the two muscle layersand hypertrophy of nerve fibers
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
VIGOROUS ACHALASIA Compared with classic achalasia vigorous achalasia has been defined as
achalasia with relatively high esophageal contraction amplitudes often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain
Defined by amplitude greater than or equal to 37 mm Hg and patients with classic achalasia defined as amplitude less than 37 mm Hg are found to have substantial overlap in radiographic parameters of esophageal dilation tortuosity and tertiary contractions
Gastroenterology 1991 Sep101(3)743-8
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Manometric properties of repetitive waves and lower esophageal sphincter pressure and clinical aspects of chest pain dysphagia heartburn and satisfactory responses to pneumatic dilation were found similar in a study by Goldenberg SP etal similar in both forms of achalasia A separate analysis of patients with mean contraction amplitude greater than 60 mm Hg revealed similar findings
It is concluded that use of amplitude as a criterion for classifying achalasia is arbitrary and of dubious value
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
PATHOGENESIS Idiopathic Infectious neurogenic degeneration Severe emotional stress Trauma Drastic weight reduction Chagasrsquo disease (parasitic infection with Trypanosoma cruzi) It is hypothesized that the aperistalsis of achalasia is secondary to
interruption of normal vagal cholinergic motor function whereas the failure of LES relaxation is due to derangement of vagal inhibitory nerves
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
There is a selective loss of postganglionic inhibitory neurons that contain both nitric oxide (NO) and VIP whereas there is sparing of the postganglionic stimulatory (cholinergic) neurons
Diminished availability of potent relaxant NO This produces a loss of inhibitory input resulting in a non relaxing
hypertensive LES
This is demonstrated by an exaggerated motor response (spasm) to injection of cholinergic agonists demonstrating a classic supersensitive response of the denervated tissue
Therefore elevated LES tone may be the consequence of disproportionate loss of inhibitory influence
In addition nitric oxide synthetase (NOS) levels in the LES muscle are considerably lower than normal
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
PSEUDOACHALASIA
Pseudoachalasia is an achalasia-like disorder that is usually produced by adenocarcinoma of the cardia
Also described in relation to benign tumours at the level of cardia and other cancers outside the oesophagus (bronchus pancreas)
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
CLINICAL FEATURES Most common in middle life but can occur at any age
Combination of three esophageal symptom complexesbull First and foremost is the insidious development of dysphagia for
both solids and liquids caused by failure of propulsive forcebull Obstruction to flowbull Incoordination of contraction and relaxation
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
About 40 of patients with achalasia complain of substernal chest pain
Gastroesophageal reflux symptoms due to an inability to adequately clear the refluxed acid
Regurgitation is frequent and there may be overspill into the trachea especially at night
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Many patients with achalasia are initially misdiagnosed with GERD
Nearly 40 of achalasia patients have some degree of heartburn due to either poor clearance of refluxed acid or from exogenous ingested acid
Weight loss although not usually excessive Regurgitation ndash previous day food Sticking of food in chest Worsen on lying down Maneuvers to allow passage of food Pulmonary complications
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
DIAGNOSIS
Patients with suspected achalasia should undergo a barium esophagram an upper endoscopy and esophageal manometry to confirm the diagnosis
(SAGES guidelines Level +++ Grade- Strong)
Achalasia may be suspected at endoscopy by finding a tight cardia and food residue in the oesophagus
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Barium radiology may show hold-up in the distal oesophagus abnormal contractions in the oesophageal body and a tapering stricture in the distal oesophagus often described as a lsquobirdrsquos beakrsquo
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Sensitivity 95 The gastric gas bubble is usually absent These typical features of well-developed achalasia are often absent
and endoscopy and radiology can be normal A firm diagnosis is established by oesophageal manometry
Classically the LOS does not relax completely on swallowing there is no peristalsis and there is a raised resting pressure in the oesophagus
The LOS pressure may be elevated but is often normal
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
The ldquoGold Standardrdquo Test- Esophageal manometry demonstrating esophageal body aperistalsis and incomplete LES relaxation
Esophagogastroduodenoscopy- Rule out pseudoachalasia due to extrinsic compression by a tumor at the level of the gastroesophageal (GE) junction Pseudoachalasia should be suspected in elderly patients when there is a short duration of symptoms and in the setting of marked weight loss
Endoscopic ultrasound may be a useful adjunct in such patients where there is a high suspicion for malignancy
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
MANOMETRY Manometry is now widely used to diagnose oesophageal motility
disorders Recordings are usually made by passing a multilumen catheter with three to eight recording transducers at different levels down the oesophagus and into the stomach
Electronic microtransducers that are not influenced by changes in patient position during the test are used
The catheter is introduced into the oesophagus and recordings are taken at intervals of 05ndash10 cm to measure the length and pressure of the LOS and assess motility in the body of the oesophagus during swallowing
High-resolution manometry uses a multiple (up to 30) microtransducer catheter with the results displayed as graphs
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
VIDEOMANOMETRY
>
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Treatment
Pharmacological Endoscopic Surgical
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
PNEUMATIC DILATION
Among non operative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications
It should be considered in selected patients who refuse surgery or are poor operative candidates (++++ strong)
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
First described by Plummer
This involves stretching the cardia with a balloon to disrupt the muscle and render it less competent
Balloons of 30ndash40 mm in diameter are available and are inserted over a guidewire
The use of esophageal stents cannot be recommended for the treatment of achalasia (++ strong)
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Perforation is the major complication With a 30-mm balloon the incidence of perforation has been less than 05 per cent
The risk of perforation increases with bigger balloons and they should be used cautiously for progressive dilatation over a period of weeks Forceful dilatation is curative in 75ndash85 per cent of cases The results are best in patients aged more than 45 years
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
VIDEOPNEUMATIC BALLOON DILATATION
>
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Historically the most popular treatment for achalasia has been by forceful pneumatic dilation
The success rate of this procedure is 55 to 70 with a single dilation but can be increased to nearly 90 with multiple dilations
However the risk of perforation with each dilation is at least 3 to 5 and has been reported as high as 12 in some series
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Furthermore when stratified by age balloon dilation is less than 50 effective in patients younger than 40 years old and is rarely effective in adolescents
In the only prospective randomized trial performed comparing balloon dilation with surgery myotomy outperformed balloon dilation with 95 to 65 results
The need for further dilations is determined by the persistence of symptoms approximately 4 weeks after treatment
The reported incidence of esophageal perforation from pneumatic dilation ranges between 04 and 5
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
BOTULINUM TOXIN
Botulinum toxin injection can be administered safely but its effectiveness is limited especially in the long term It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++ strong)
Less effective than balloon dilation and requires retreatment to maintain an efficacy rate of 65
Botox injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30) during dissection later
Aiding in the diagnosis of patients- A good response to Botox is usually an indication that the patient will have long-term relief following surgical myotomy
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
PHARMOLOGICAL MANAGEMENT
SAGES Guidelines (++++ strong)
ndash Limited rolendash Very early stages of the diseasendash Temporarily prior to more definitive treatmentsndash Patients who fail or are not fit for other treatment
modalities
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Nitrates Sildenafil Drugs such as calcium channel antagonists have been used but are
ineffective for long-term use However sublingual nifedipine may be useful for transient relief of
symptoms if definitive treatment is postponed Limited effectiveness Inconsistent absorption Tolerance Side effects Headache and hypotension
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
SURGICAL MANAGEMENT Esophagocardiomyotomy was introduced by Ernest Heller in 1914 and
the operation was performed through the abdomen Most effective long-term treatment for achalasia When properly performed a Heller myotomy can be expected to result in
permanent relief of dysphagia in 85 to 100 of patients
In 1958 Ellis et al described the transthoracic approach The success rate at relieving dysphagia was approximately 90 whether
cardiomyotomy was performed through a thoracotomy or laparotomy Esophageal perforation incidence- Less than 1
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
SAGES Recommendations on Myotomy
Laparoscopic myotomy with partial fundoplication provides superior and longer lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++ strong)
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity but the literature is inconclusive (++ strong)
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2cm on the stomach (+ weak)
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure performed only when the myotomy is done transabdominally
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++ strong)
The optimal type of fundoplication is debated (posterior vs anterior) but partial fundoplication should be favored over total fundoplication as it is associated with decreased dysphagia rates and similar reflux control (++ weak)
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++ weak)
Endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++ weak)
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++ strong)
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+ weak)
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
MYOTOMY OF THE LOWER ESOPHAGEAL
SPHINCTER (HELLER MYOTOMY) Lenght of myotomy
ndash 6 ndash 7 cmndash Extending 05- 1 cm onto the cardia of stomach
When performed adequately (ie reducing sphincter pressure to lt10 mmHg) and done early in the course of disease LES myotomy results in symptomatic improvement with the occasional return of esophageal peristalsis
The major complication is gastroesophageal reflux and most surgeons
therefore add a partial anterior fundoplication (Heller-Dorrsquos operation) or Toupet Fundoplication
It is successful in more than 90 per cent of cases
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Following successful esophageal myotomy patients may continue to
have minor symptoms of dysphagia which is attributable to esophageal aperistalsis This may lead to inadequate clearance of refluxed acid
The type of fundoplication is important in order to avoid significant obstruction In general a 360-degree fundoplication should not be used because it will be too obstructive in this setting
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
But the role for adding an antireflux procedure to the esophagomyotomy remains controversial
Proponents base their recommendations on the high reported incidence of gastroesophageal reflux and esophagitis following esophagocardiomyotomy
Conversely there are those who have demonstrated successful relief of dysphagia with a low incidence of reflux esophagitis following Heller myotomy and believe that an antireflux procedure is unnecessary
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Concomitant Antireflux Procedure NissenToupetDor
Toupet or Dor fundoplication preferred over a 360-degree Nissen fundoplication Controversy exists as to whether a full 360-degree Nissen fundoplication increases the rate of dysphagia compared with an incomplete fundoplication
Toupet and Dor fundoplications Variable outcomes Toupet- Long term reflux control Dor fundoplication- Less disruption of hiatal anatomy and fundal
coverage of the esophageal mucosa after myotomy There is a hypothetical advantage of using the two limbs of the
fundoplication to hold the edges of the myotomy apart In cases with concern regarding the integrity of the mucosa after myotomy Dor repair allows fundal coverage of the esophageal mucosa
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Further it can be argued that the antireflux procedure is contraindicated because of the outflow resistance produced in the presence of esophageal aperistalsis
Even small increases in LES pressure may result in long-term failure of the procedure due to progressive esophageal dilation and diminished propulsion
A recent prospective randomized study by the group at Vanderbilt University demonstrated that Heller myotomy plus a Dor anterior fundoplication is superior to Heller myotomy alone with regard to the incidence of postoperative reflux as measured by 24-hour pH testing
Richard et al study on post op GERDndash Dor fundoplication reduced GERD from 476 to 9
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Laparoscopic Esophagomyotomy
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
After the lower esophagus anterior vagus nerve and cardia of the stomach are exposed the gastroesophageal fat pad is excised
The myotomy is then carried out utilizing blunt dissectors a scissors or hook without electrocautery or an ultrasonic shears
The myotomy is begun on the esophagus and carried proximally a distance of 5 to 6 cm from the GE junction and distally 05 to 1 cm onto the cardia
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
The myotomy is guided by intraoperative endoscopy to ascertain that it is carried at least 1 cm beyond the squamocolumnar junction
Surgery relieves symptoms in 70 to 90 percent of people
Symptom relief is sustained in about 85 percent of people 10 years after surgery and in about 65 percent of people 20 years after the surgery
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
VIDEOHELLER MYOTOMY
>
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Complications of Myotomy
Esophageal perforation during surgery has been reported to occur on average in 69 of patients but with clinical consequences in only 07 of patients
In one series of 222 patients inadvertent esophagotomy occurred in 16 (72) resulting in longer hospitalization but not different postoperative symptomatology
Several studies have suggested an increased risk for intraoperative complications during esophagomyotomy after prior endoscopic intervention
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Per-Oral Endoscopic Myotomy Submucosal injection of saline solution at the midesophageal level
approximately 13 cm from the gastroesophageal junction A standard endoscope with a cap is introduced into the submucosal layer
through a 2-cm longitudinal incision on the mucosal surface A submucosal tunnel is then created and extended to 3 cm onto the stomach with a triangular tip knife and spray coagulation with direct endoscopic vision or blunt dissection with the flexible endoscope or standard balloon dilation
Dissection of the circular muscle begins approximately 7 cm from the gastroesophageal junction with spray coagulation and a triangular tip knife Division of the muscle continues for approximately 2 cm distal to the gastroesophageal junction narrowing is typically seen endoscopically during passage through the LES
After myotomy smooth passage of the endoscope through the gastroesophageal junction should be seen The site of entry in the mucosa is then closed with hemostatic clips or fibrin glue
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
DIFFUSE OESOPHAGEAL SPASM
Originally described by Osgood in 1889 It is found in 3- 5 of patients evaluated for esophageal motility disorders Diffuse oesophageal spasm is a condition in which there are
incoordinate contractions of the oesophagus causing dysphagia andor chest pain
Diffuse esophageal spasm is characterized by rapid progression of abnormally high amplitude longer duration contractions down the esophagus and aperistalsis during more than 20 of wet swallows
In diffuse esophageal spasm manometric abnormalities may be found over the total length of the esophagus but is usually confined to the distal two thirds
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
The condition may be dramatic with spastic pressures on manometry of 400ndash500 mmHg marked hypertrophy of the circular muscle and a corkscrew oesophagus on barium swallow
Prolonged ambulatory oesophageal manometry that correlates episodes of chest pain with manometric abnormalities may establish the diagnosis
Patients are often hypersensitive to provocative agents such a cholinergic agonists and pentagastrin
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Barium esophagogram may be normal or may show disorganized peristalsis with the classic corkscrew esophagus Sensitivity- 71 Upper endoscopy is typically unremarkable
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Exact etiology is unknown Recent evidence suggests that there may be a defect in neural
inhibition that is normally mediated by nitric oxide within the esophageal body
Surgery plays a very limited role in the treatment of this problem
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
24-hour ambulatory motility studies allow for the calculation of a composite score for more accurate diagnoses of the disease
Patients with segmental or diffuse esophageal spasm are at risk for developing an epiphrenic or midesophageal diverticulum
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
TREATMENT
There is no proven pharmacological or endoscopic treatment Calcium channel antagonists vasodilators and endoscopic dilatation
have only transient effects Sometime in patients with intolerable pain and dysphagia causing
malnutrition extended oesophageal myotomy up to the aortic arch may be required
Surgical treatment of diffuse spasm is more successful in improving dysphagia than chest pain and caution should be exercised in patients in whom chest pain is the only symptom
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
The initial treatment of patients with diffuse esophageal spasm should include medical therapy and lifestyle adjustments
Indications for operative treatment are not as clear as in achalasia and should only be carried out by surgeons with considerable experience with the technique of long esophagomyotomy
The presence of chest pain alone is not an indication for operative intervention
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Long esophageal myotomy is indicated for dysphagia in a patient with documented diffuse esophageal spasm and whose symptoms are not relieved by medical therapy
The technique of long myotomy is carried out via a thoracoscopic approach carrying the myotomy over the entire distance of the manometric abnormality
The presence of an epiphrenic or midesophageal diverticulum complicates the procedure requiring dissection of the neck of the diverticulum and division via an endoscopic linear stapler
The overlying muscle is reapproximated with permanent sutures and then the myotomy is performed on the opposite esophageal wall
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
NUTCRACKER OESOPHAGUS First described in 1977 by Pope and associates The term was coined by Benjamin and Castell based on the
characteristic high-amplitude (gt180 mm Hg) waves that may be prolonged in nature (gt6 s)
Nutcracker oesophagus is a condition in which peristaltic pressures of more than 180 mmHg developes
It is said to cause chest pain but there is still some debate as to whether it is a
real motility disorder Defining peristaltic amplitude has been debated should be
increased to 260 mm Hg which is more likely to be associated with chest pain and dysphagia
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
As the pattern of peristalsis is not altered barium esophagogram is usually unremarkable
Upper endoscopy is normal but endoscopic ultrasonography may demonstrate thickening of the muscularis propria
Psychiatric disorders such as anxiety depression and somatoform disorders appear to be found in higher-than-predicted frequencies in this population as first described by Clouse and associates
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
AUTOIMMUNE DISEASESEsophageal involvement is mainly seen in Systemic sclerosis Polymyositis Dermatomyositis Systemic Lupus Erythematosus Polyarteritis nodosa or rheumatoid disease While most involve weak peristalsis swallowing difficulties may
be compounded by pharyngeal problems in the disorders that primarily affect skeletal muscle (eg polymyositis) or extraoesophageal problems such as involvement of the cricoarytenoid joint in rheumatoid disease or dry mouth in Sjoumlgrenrsquos syndrome In systemic sclerosis smooth muscle atrophy causes hypoperistalsis
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
HYPERTENSIVE LES Hypertensive lower esophageal sphincter is diagnosed when there
is an elevated LES pressure (gt45 mm Hg) in the absence of a concomitant disorder of esophageal body peristalsis
Primary disorder of esophageal motility characterized by a resting pressure in the LES that exceeds the upper limit of normal while esophageal body peristalsis is normal
The most common symptoms are dysphagia and chest pain
However in a certain subset of patients acid reflux may accompany the hypertensive LES with symptoms of GERD
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
The LES is involved leading to a loss of the antireflux barrier - GERD
A wide range of symptoms - Mild to severe dysphagia accompanied by regurgitation and aspiration
Reflux can be severe and is exacerbated by weak acid clearance so that strictures can occur
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
MANAGEMENT There are no drugs that specifically correct the motor disorder and
medical treatment is mainly directed at minimising reflux-induced damage with PPIs A small number of patients may require antireflux surgery
When hypertensive LES is associated with GERD andor a type III hiatal hernia A laparoscopic myotomy with partial fundoplication is indicated for isolated hypertensive LES
Laparoscopic Nissen fundoplication should be performed in patients with hypertensive LES with GERDtype III hiatal hernia
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
EOSINOPHILIC ESOPHAGITIS Eosinophilic oesophagitis is a disorder that occurs either alone
or as a manifestation of eosinophilic gastroenteritis
Eosinophilic esophagitis (EE) was first described in 1977
It is characterised by eosinophilic infiltration of the oesophageal wall presumably of allergic or idiopathic origin
The most common presenting symptom is dysphagia and more than half have some history of atopy
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
TREATMENT Symptomatic
Testing for food allergies and elimination of identified items from the diet
Second line therapy includes inhaled or ingested corticosteroids
Immunotherapy directed against interleukin (IL)-5 which has a major role in eosinophil recruitment is a promising innovative approach
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
If dysphagia is not relieved with steroids it may be necessary to dilate the esophagus
Great care must be exercised as the inflamed EE is quite friable The mucosa tears easily and esophageal perforation (full thickness laceration) has been reported with EE dilation so should be used with caution and only when the above therapies fail
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Pharyngeal Esophageal Diverticula
Most oesophageal diverticula are pulsion diverticula that develop at a site of weakness as a result of chronic pressure against an obstruction
Symptoms are mostly caused by the underlying disorder unless the diverticulum is particularly large
Those found proximally and distally in the esophagus are usually pulsion diverticula caused by elevated intraluminal pressures
These are not true diverticula as they are comprised only of mucosa
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Esophageal diverticula are uncommon Can be found at any level of the esophagus but generally classified in
three groups
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Midesophageal diverticula are typically traction diverticula resulting from an extrinsic process which occurs adjacent to the esophageal wall pulling on it
These are true diverticula commonly found near the carina
Mostly result from tubercular inflammations and do not usually require surgical treatment
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Zenkers Diverticulum
Late middle aged or elderly
Acquired condition
Most common types of esophageal diverticula
Thought to arise from increased pressure within the pharynx
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Not really an oesophageal diverticulum as it protrudes posteriorly above the cricopharyngeal sphincter through the natural weak point (the dehiscence of Killian) between the oblique (thyropharyngeus) and horizontal (cricopharyngeus) fibres of the inferior pharyngeal constrictor
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Manometric evaluation of the UES shows some degree of lack of coordination between bolus propagation and sphincter relaxation results in increased pressure in the pharynx in more than two thirds of patients
There is also evidence suggesting that the muscles of the cricopharyngeus and the proximal esophagus are fibrosed and therefore do not function normally
Histological analysis of sphincter muscles have shown them to be replaced by fibrotic tissue possibly explaining the abnormal response of the sphincter to swallowing
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Dysphagia and regurgitation are the most common complaints
Dysphagia may occur as a result of the dysfunctional UES It may also be due to displacement or obstruction of the
pharyngoesophageal junction caused by a large diverticulum Aspiration halitosis excessive salivation and a lump in the
throatldquo
Physical exam is usually not helpful but may reveal a palpable mass
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
A contrast esophagogram should be obtainedEsophagoscopy ManometryA 24 hour pH study can be done to determine if
there is associated gastroesophageal reflux
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
TREATMENT
Endoscopically with a linear cutting stapler to divide the septum between the diverticulum and the upper oesophagus producing a diverticulo-oesophagostomy
Or by open surgery involving pouch suspension (diverticulopexy) Myotomy of the cricopharyngeus All techniques have reported good results
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
EPIPHRENIC DIVERTICULA
Pulsion diverticula situated in the lower oesophagus above the diaphragm
May be asymptomatic or may present with dysphagia regurgitation chest pain halitosis chronic cough aspiration pneumonia or upper gastrointestinal bleeding
Mondiere made the first attribution of esophageal dysmotility as the cause of esophageal diverticula in 1833
Due to loss of coordination between an incoming pressure wave and appropriate relaxation of the LES
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Not true diverticula as they consist of only esophageal mucosa and submucosa herniating through the muscular wall of the esophagus
Large diverticula may be excised and this should be combined with a myotomy from the site of the diverticulum down to the cardia to relieve functional obstruction
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
INVESTIGATIONS
Upper endoscopy- Information about the neck of the diverticulum and other anatomic information that might be helpful in deciding the surgical approach
Rules out any contributing functional obstruction or possible malignancy
24-Hour pH testing may demonstrate gastroesophageal reflux
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
MANAGEMENT The treatment in these cases is directed at the underlying pathophysiology
and the functional obstruction of the distal esophagus as well as dealing with the diverticulum Surgical therapy is generally necessary for epiphrenic diverticulum
The surgical approach has been a diverticulectomy via a left thoracotomy with or without an associated esophageal myotomy
The diverticulum is dissected free and then resected with a stapler while a bougie of appropriate size is kept within the lumen of the esophagus to prevent narrowing
Postoperatively a contrast study is obtained to rule out a leak
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
DIVERTICULECTOMY versus DIVERTICULOPEXY
To avoid infection due to leak from a resected diverticulum some surgeons perform a diverticulopexy
The diverticulum is sutured to the precervical fascia so that the apex is cephalad to its neck without opening the mucosa
In a nonrandomized study of 43 patients approached by a transcervical route
- 14 had diverticulectomy and- 29 had diverticulopexy
Two patients in both groups developed neck infections and one patient in the diverticulectomy group developed mediastinitis There was no statistical difference in the incidence of wound infection or mediastinitis between the two groups
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
A rare condition in which there are multiple tiny outpouchings from the lumen of the oesophagus
The pseudodiverticula are dilated excretory ducts of oesophageal sebaceous glands
It is questionable whether the condition produces any symptoms or requires any treatment
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
CRICOPHARYNGEAL BAR
A cricopharyngeal bar is identified radiographically by a persistent posterior indentation of contrast
Best seen on a lateral view during a videoesophagogram
Residual contrast may be seen above the cricopharyngeus well after the closure of the UES
No consistent clinical symptoms are usually identified in these patients
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Exact etiology and the significance of a cricopharyngeal bar are unknown
But the persistent indentation has been speculated to result from either a failure to relax or an incoordination of the constrictors and the cricopharyngeus muscle
Also called as UES achalasia
Dantas and associates investigated six patients with cricopharyngeal bars and eight controls without any finding on barium swallow
The subjects were assessed using videofluoroscopy and UES manometry The patients were found to have normal contraction of the pharynx normal UES pressures and normal bolus transit When compared to the controls the patients with cricopharyngeal bars had reduced UES relaxation and increased upstream bolus pressures This suggests that cricopharyngeal bars arise as a result of failure of the muscle to completely relax
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
In contrast others have found that resting relaxation and contraction pressures are normal in patients with cricopharyngeal bar
These authors claim that the cricopharyngeal muscle is the only normal portion of the pharyngoesophageal segment and that the inferior constrictor and the proximal esophagus are abnormally dilated
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Surgical intervention for cricopharyngeal bar is not needed except for very selected patients
The patients should not have an underlying medical condition known to affect motility (myopathy Parkinsons disease or myasthenia gravis)
Neoplasia must be excluded and gastroesophageal reflux should be eliminated as a cause of the cricopharyngeal bar
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Resolution of dysphagia and regurgitation may be expected and has been reported in 82- 100 of patients treated with this approach
Mortality from this procedure should be less than 1 The most disturbing complication esophagocutaneous fistula occurs
in 6- 20 of cases but heals spontaneously
Other potential complications include soft tissue infection mediastinitis recurrent laryngeal nerve injury hematoma and late stenosis
The frequency of these complications ranges between 1 and 6
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Manometric findings that do not fit into any classic patterns are placed in the category of nonspecific esophageal motor disorders
Pathogenesis of NEM is multifaceted and has no single isolated cause
Several collagen vascular disorders are known to cause abnormalities of esophageal motility including scleroderma dermatomyositis polymyositis and lupus erythematosus
All affect the neuromuscular esophageal architecture resulting in poor esophageal motility
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Diagnostic tests include barium esophagraphy and manometric studies
Esophagraphy is helpful to rule out disorders with defined abnormalities and identifies abnormal esophageal body contractions as well as abnormalities of the LES
Manometry is critical to identify abnormal esophageal body contractions as well as abnormalities of the LES
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
Treatment of NEM is difficult because a primary diagnosis is evasive
Those with collagen vascular or neuromuscular disorders are treated for their primary medical condition which often results in improved esophageal motility
For those whose underlying condition remains undiagnosed combination therapy including medications and therapeutic interventions can be applied as guided by the prevailing manometric findings
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
SCLERODERMA
Scleroderma is a systemic disease accompanied by esophageal abnormalities in approximately 80 of patients
Small vessel inflammation Subsequent perivascular deposition of collagen Vascular compromise Smooth muscle atrophy
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus with absent peristalsis in the distal smooth muscle portion
The surgical management is somewhat controversial but the majority of opinions suggest that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice
Only 50 of the patients have a good-to-excellent result
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
Slide 53
Slide 54
Concomitant Antireflux Procedure NissenToupetDor
HELLER MYOTOMY WITH TOUPET FUNDOPLICATION
Slide 57
Laparoscopic Esophagomyotomy
Slide 59
Slide 60
Slide 61
Slide 62
VIDEO HELLER MYOTOMY
Complications of Myotomy
Per-Oral Endoscopic Myotomy
DIFFUSE OESOPHAGEAL SPASM
Slide 67
Slide 68
Slide 69
Slide 70
Slide 71
TREATMENT
Slide 73
Slide 74
Slide 75
NUTCRACKER OESOPHAGUS
Slide 77
AUTOIMMUNE DISEASES
HYPERTENSIVE LES
Slide 80
MANAGEMENT
EOSINOPHILIC ESOPHAGITIS
Slide 83
Slide 84
Slide 85
TREATMENT (2)
Slide 87
Pharyngeal Esophageal Diverticula
Slide 89
Slide 90
Zenkers Diverticulum
Slide 92
Slide 93
Slide 94
Slide 95
Slide 96
TREATMENT (3)
EPIPHRENIC DIVERTICULA
Slide 99
INVESTIGATIONS
MANAGEMENT (2)
Slide 102
Slide 103
Slide 104
DIVERTICULECTOMY versus DIVERTICULOPEXY
DIFFUSE INTRAMURAL PSEUDODIVERTICULOSIS
CRICOPHARYNGEAL BAR
Slide 108
Slide 109
Slide 110
Slide 111
NONSPECIFIC ESOPHAGEAL MOTOR DISORDERS
Slide 113
Slide 114
SCLERODERMA
Slide 116
Slide 117
THANK YOU
Subject seminar motility disorders of esophagus
ANATOMY
GRADUATION OF STRIATED TO SMOOTH MUSCLE FIBERS
MICROSTRUCTURE
Slide 5
Slide 6
Areas of weakness
Slide 8
Slide 9
MYENTERIC PLEXUS
Function of the Myenteric Plexus in Peristalsis
Slide 12
NEURAL CONTROL OF LES
MOTILITY DISORDERS OF ESOPHAGUS
Slide 15
Slide 16
Slide 17
ACHALASIA
ACOG DEFINITION OF ACHALASIA
Slide 20
VIGOROUS ACHALASIA
Slide 22
PATHOGENESIS
Slide 24
PSEUDOACHALASIA
CLINICAL FEATURES
Slide 27
Slide 28
DIAGNOSIS
Slide 30
Slide 31
Slide 32
Slide 33
MANOMETRY
Slide 35
VIDEO MANOMETRY
Slide 37
PNEUMATIC DILATION
Slide 39
Slide 40
VIDEO PNEUMATIC BALLOON DILATATION
Slide 42
Slide 43
BOTULINUM TOXIN
Complications
PHARMOLOGICAL MANAGEMENT
Slide 47
SURGICAL MANAGEMENT
SAGES Recommendations on Myotomy
Slide 50
Treatment Options After Failed Myotomy
MYOTOMY OF THE LOWER ESOPHAGEAL SPHINCTER (HELLER MYOTOMY)
![BMC Gastroenterology BioMed Central - link.springer.com · Achalasia of the esophagus (AE) is a motility disorder of controversial etiology [1–4]. Myenteric plexus degenera-tion](https://static.fdocuments.us/doc/165x107/5e189a643b123f130f15d563/bmc-gastroenterology-biomed-central-link-achalasia-of-the-esophagus-ae-is-a.jpg)
