DYSPHAGIA

DYSPHAGIA- a Greek word that means disordered eating

OUTLINEAnatomy of the EsophagusOverview of DysphagiaInvestigation - History - Physical Examination - Special InvestigationsCategorization of Causes of Dysphagia - Neuromuscular Causes

Anatomy of the esophagusA muscular canal extending from the pharynx to the stomach. - about 23 25 cm in lengthBegins in the neck at the lower border of the cricoid cartilage (opposite C6) and ends at the cardiacorifice of the stomach (opposite T11).Pierces the diaphragm slightly to the left of the midline. - enters the stomach on its right side after a short course of about 1.25cmDeeply placed as it lies behind the left lobe of liver.

Is the narrowest part of the digestive tube.Most contracted at its commencement, and at the point where it passes through the diaphragm.Has 3 constrictions in its vertical course: (i) where the esophagus commences at the cricopharyngeal sphincter (ii) where it is crossed by the aortic arch and left mainbronchus (iii) where it pierces the diapraghm (Lower esophageal sphincter, LES is located here as well)

Blood SupplyCervical portion : Inferior thyroid arteryThoracic portion : Bronchial & esophageal branches of thoracic aortaAbdominal portion : Ascending branches of the left phrenic and left gastric arteries

Nerve SupplyDerived from the vagi and from the sympathetic trunks. - Form a plexus which are groups of ganglion cells.

DysphagiaRefers to difficulty in eating as a result of disruption in the swallowing process. - the feeling that food or liquid is stuck in the throat or at any point before the food enters the stomachMay be experienced when swallowing solid foods, liquids, or both.Disorders leading to dysphagia may affect the oral, pharyngeal, or esophageal phases of swallowing.Can strike at any age, although the risk increases with age.Divided into : (i) Oropharyngeal dysphagia (Mouth Upper esophagus) (ii) Esophageal dysphagia (Through esophagus Stomach)

Signs & Symptoms

Symptoms of oropharyngeal dysphagia: - Difficulty trying to swallow - Choking or breathing saliva into your lungs while swallowing - Coughing while swallowing - Regurgitating liquid through your nose - Breathing in food while swallowing - Weak voice - Weight loss

Symptoms of esophageal dysphagia: - Pressure sensation in your mid-chest area - Sensation of food stuck in your throat or chest - Chest pain - Pain with swallowing - Chronic heartburn - Belching - Sore throatCan be a serious threat to ones health due to the risks of : - Aspiration pneumonia - Malnutrition - Dehydration - Weight loss - Airway obstruction Thorough history taking and careful physical examination are important in the diagnosis and treatment of dysphagia.

investigationHistory Subjective site of obstruction is not always exact. - Patient often merely points vaguely to behind the sternum.Diagnosis may be made based on : (a) History of past swallowed caustic materials. (b) History of reflux esophagitis - Suggests peptic stricture (c) Patient with achalasia (failure of LES to relax) - Tends to be young - Usually without loss of weight (d) Malignant stricture - Has a short history - Usually occurs in the elderly - Is associated with weight loss

ExaminationOften negative.However, search is made for : (a) Plummer-Vinson syndrome - Smooth tongue - Anaemia - Koilonychia (b) Secondary nodes from an esophageal carcinoma - Can be felt in the neck and supraclavicular fossae. (c) Carcinoma of the cardia (stomach) - Upper abdomen is palpated carefully. - Common cause of dysphagia in the elderly. - More common than esophageal CA.

Koilonychia

Special investigationsBarium swallow - May demonstrate : (i) the characteristic appearance of a cervical web (ii) extrinsic compression and dilated esophagus of achalasia

Fibreoptic esophagospy - Enables biopsies to confirm malignancies - Permits therapeutic dilatation or intubation (if indicated).

* The investigations are complementary.

Barium swallow testshowing dilated esophagus in achalasia

Fibreoptic esophagoscopy

Causes of dysphagia

Neuromuscular Stroke Myasthenia gravis Poliomyelitis Guillain-Barre Neuropathy Multiple sclerosis Bulbar palsy

Extraluminal Presence of enlarged lymph nodes Thoracic aortic aneurysm Bronchial carcinoma Retrosternal goitre

Mural Congenital atresia Inflammatory stricture Caustic stricture Achalasia Plummer-Vinson syndrome Shattzki Ring Tumour of esophagus or cardia

Intraluminal Foreign body Food bolus

NEUROMUSCULAR CAUSES

Stroke - Characterized by the sudden loss of blood circulation to an area of the brain ~ results in corresponding loss of neurologic function - Previously called cerebrovascular accident (CVA) or stroke syndrome - Classified as either haemorrhagic or ischaemic. (i) Acute ischaemic stroke (80-87%): - Stroke is caused by thrombosis or embolism. - Results from events that limit or stop blood flow. - More common than haemorrhagic stroke. (ii) Haemorrhagic stroke (13-20%): - Patients present with similar neurologic deficits BUT tend to be more ill than ischaemic stroke patients. - Can be intracerebral (more common) or subarachnoid.

* Middle cerebral artery (MCA) is the most commonly affected vessel in CVA.

* Transient ischaemic attack (TIA) - Like a temporary ischaemic attack. - An artery is temporarily blocked; prevents blood flow to part of the brain ~ causes that part of brain to stop functining - Symptoms are similar to ischaemic stroke. ~ most last less than 30 minutes - TIA patients are at HIGH risk of developing stroke soon thereafter. ~ immediate medical evaluation is needed.

Clinical Features: - Symptoms depend on part of brain that is damaged. ~ Develop suddenly and without warning/ On and off for first day or two. ~ Most severe when stroke first happens/ Can slowly get worse.

- Symptoms: (i) Headache (if stroke caused by bleeding in brain) (ii) Change in alertness, hearing and taste (iii) Confusion or loss of memory (iv) Dysphagia (v) Dizziness or abnormal feeling of movement (vertigo) (vi) Loss of balance and coordination (vii) Muscle weakness in the face, arm, or leg (usually just on one side) (viii) Numbness or tinglingon one side of the body (ix) Trouble speaking or understanding others who are speaking (x) Trouble walking (xi) Lack of control over the bladder or bowels

Myasthenia gravis - A type of autoimmune disorder of the neuromuscular junction (NMJ) characterised by muscle weakness. - Can present at any age; has a predilection for women. - Exact cause is unknown. Pathophysiology: - Due to presence of autoantibodies against acetylcholine receptors (AchR) ~ leads to loss of functional AchRs at NMJ either by: (i) increasing the internalization and degradation of the receptors (ii) blocking the binding of Ach to its receptors

- Results in a characteristic pattern of progressively reduced muscle strength with repeated use ~ recovery of muscle strength only after a period of rest Clinical Features: - Weakness typically first noticed in extraocular muscles: (i) Drooping eyelids (Ptosis) (ii) Double vision (Diplopia)

- Generalised muscle weakness can fluctuate dramatically ~ alterations over course of days, hours or even minutes ~ affected muscles include those which control breathing (SOB), swallowing (dysphagia), facial expressions and limb movements.

Poliomyelitis - A viral disease that can affect nerves and can lead to partial or fullparalysis ~ caused by small RNA viruses of the enterovirus group of the Picornavirus family.

Pathphysiology: - Poliovirus is spread by the fecal-oral route and by aerosol droplets. ~ shed in oral secretions (for several weeks) ~ shed in faeces (for several months) - Poliovirus destroys the anterior horn cells in the spinal cord.

Clinical Features: - 3 basic patterns of polio infection: (i) Subclinical infections ~ Includes malaise, headache, vomiting, slight fever and sore throat. (ii) Non-paralytic ~ Includes back pain, diarrhea, fatigue etc. (iii) Paralytic ~ Includes dysphagia, difficulty in breathing, muscle contractions (calf, neck or back), abnormal sensations etc.

Guillain-Barr syndrome (GBS) - One of the most life-threatening diseases of peripheral nervous system. - A collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes. - May develop spontaneously or after a systemic infection (usually viral) or other stress.

Pathophysiology: - Is a postinfectious, immune-mediated disease. - Cellular and humoral immune response play a role in its development.

Lymphocytic infiltration of spinal roots and peripheral nerves

Macrophage-mediated, multifocal stripping of myelin

Defects in the propagation of electrical nerve impulses

Eventual absence or profound delay in conduction

Flaccid paralysis

* Recovery is usually associated with remyelination.

Clinical Features: - Patients usually present with rapidly progressive, ascending motor weakness. ~ may lead to death from failure of respiratory muscles. - Sensory involvement usually much less striking as compared to motor dysfunction.

* With supportive care, most affected individuals recover over time.

Neuropathy - Damage to a single nerve or nerve group, which results inloss of movement, sensation, or other function of that nerve. - A type of damage to nerves outside the brain and spinal cord (peripheral neuropathy). - Most commonly caused by injury. ~ Systemic disorders can also cause isolated nerve damage.

Pathophysiology: - Can be caused by long-term pressure on a nerve due to swelling or injury ~ myelin sheath or axon of nerve may be damaged ~ slows or prevents signals from travelling through the damaged nerves

Clinical features: - Symptoms depend on the specific nerve affected and may include: (i) Loss of sensation (ii) Paralysis (iii) Tingling, burning, pain,abnormal sensations (iv) Weakness

Multiple Sclerosis - An autoimmune demyelinating disorder. - Characterised by distinct episodes of neurologic deficits, separated in time, attributable to white matter lesions that are separated in space. - Becomes clinically apparent at any age; onset in childhood or after the age of 50 is relatively rare. - Twice more common in women compared to men.

Pathophysiology: - Caused by a combination of environmental and genetic factors: ~ results in a loss of tolerance to self-proteins (myelin antigens)

- Due to T cell-mediated delayed type hypersensitivity reaction towards myelin proteins. - Toxic effects of lymphocytes, macrophages and their secreted molecules may be involved in initiating axonal injury: ~ sometimes leading to neuronal death.

Clinical Features: - Multiple episodes of new symptoms (relapses) followed by episodes of recovery (remission). ~ typically, recovery is not complete ~ leads to gradual accumulation of neurologic deficits - Frequent initial manifestation: ~ Unilateral vision impairment occurring over course of a few days

- Classic MS symptoms include sensory loss, spinal cord symptoms, cerebellar symptoms etc. - May present with many other manifestations, including the following: (i) Aphasia or dysphasia (ii) Seizures (5% of patients with MS) (iii) Other paroxysmal symptoms (eg, ataxia, akinesia, paresthesias, pruritus) (iv) Significant motor complaints without sensory deficits or dysautonomia

Multiple sclerosis

Bulbar Palsy -Refers to impairment of function of thecranial nervesIX, X, XI and XII: ~ which occurs due to alower motor neuronlesion either at nuclear or fascicular level in themedulla oblongataor from lesions of the lower cranial nerves outside thebrainstem. - Is an assortment of signs and symptoms.

Causes: (a) Genetic (e) Malignancy (b) Vascular (f) Toxic (c) Degenerative diseases (g) Autoimmune (d) Inflammatory/Infective

Clinical Features: - Among some of the symptoms include: (i) dysphagia(difficulty in swallowing) (ii) difficulty in chewing (iii) nasal regurgitation (iv) slurring of speech

* Ocular muscles are spared and this differentiates it from myasthenia gravis.

-Perforation of esophagus

-Intraluminal causes

-Extraluminal causes

Perforation of the esophagusCauses:Swallow foreign bodiesRupture at esophagoscopyRupture during esophageal echocardiographyBoerhaave syndrom (spontaneous rupture)p/w - pain in the neck, chest, upper abdomen - dysphagiaBoerhaave syndromsudden rise in intraluminal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle to relax. Hx - a middle-aged man - repeated episodes of retching and - recent excessive dietary and alcohol intake.

Investigation : - chest X ray (gas in the neck and mediastinum) - Gastrograffin swallow (confirm position)

Boerhaave syndrome.Chest radiographs show pneumomediastinum (arrows).Esophagram with extravasated water soluble contrast material in left hemithorax (asterisk)

Spontaneous oesophageal perforation. Sudden onset of pain during a meal.

(a) Water soluble contrast swallow shows an ovoid filling defect (upper border shown by large arrow) due to intramural haematoma, and linear collection of submucosal contrast (small arrow).

Treatment- Medical or Surgical

Medical TherapyStandard therapy includes the following:

Admission to medical/surgical ICU Nothing by mouth Parenteral nutritional support Nasogastric suction Broad-spectrum antibiotics Narcotic analgesicsDecision depend on : Time delay in presentation and diagnosis- Extent of perforationOverall medical condition of the patient

Features that support conservative therapy include the following:

- no signs of infection - Contained perforation in the mediastinum and the visceral pleura without penetration to another body cavity - Perforation draining back into the esophagus

Surgical treatment

Tube thoracostomy (Drainage with a chest tube or operative drainage alone)Primary repair with reinforcement with pleura, intercostal muscle, diaphragm, pericardial fat, pleural flapDiversionDiversion and exclusionEsophageal resectionThoracoscopic repairEsophageal stent

Intraluminal causes

Foreign Bodies

- Previous history of swallowing of foreign object- Children (esp sharp and irregular item)- p/w - dyspahgia - coughing - dysphagia even to saliva - chocking - vomiting - retching - neck and/or throat pain

- Foreign body at the level of upper oesophageal sphincter - children lower oesophageal sphincter - adult

Investigation- X-ray - Barium Swallow

Treatment- Oesphagoscopic removal- oesophagotomy

Anteroposterior chest radiograph depicts a penny at the thoracic inlet of a 13-month-old infant who refused to eat.

ExtraluminalEnlargement of the structure surrounding esophagus that produces a mass effect compressing on the esophagus

EX :

Mediastinal lymphadenopathyBronchial carcinomaRetrosternal goitreAortic aneurysm

Cross section of the neck

Transverse section : T4 - 5

Mediastinal Lymphadenopathy

- Mediastinal nodes drain lungs, heart, thymus, and thoracic esophagus.

p/w - cough, wheezing, dysphagia, hemoptysis, atelectasis, and the obstruction of the great vessels.Mediastinum Superiorly to thracic ouletInferiorly to diaphragmAnteriorly to sternum Posterirly to vertebral column

Lymphadenopathy enlargement of lymph node

Causes - 95% by tumors or cysts. - Lymphomas and acute lymphoblastic leukemia are the most common etiologies and usually involve the anterior mediastinum - rarely infection

Investigation

Lab studies - peripheral blood smear - full blood count - hepatic and renal function, urine analysis

Imaging studies - Chest X-ray - chest CT

Treatment - underlying etiology - antibiotic - infection - surgical removal of affected node

Bronchial carcinoma p/w - cough, haemoptysis, breathlessness, wheeze and stidor - persistent respiratory infection - dysphagia

Investigation - X- ray - CT

Treatment - Surgery, chemotherapy, radiotherapy

Retrosternal goitre

- Enlargement of the thyroid gland - Physiological - Pathologicalp/w swelling in the neck, dyspnea, dysphagia, stridor, plethora or hoarseness of voiceSigns and symptoms of hyper/hypothyroidism

InvestigationThyroid function testUltrasound (size, nodularity, consistency)CT (size, effect of the thyroid gland on nearby structures)MRIBarium (esophageal obstruction)

(black arrows) large mass of low attenuation in right lobe of thyroid with (white arrow)focal bulging in mucosal portion of trachea.

Aortic AneurysmAneurysm localized or diffuse dilation of an artery with a diameter at least 50% greater than the normal size of the artery

AA - TA - AAA (most common) - TAA

media layer consist collagen and elastin.Elastic fiber fragmentation and loss with degeneration of the media result in weakening of the aortic wall, loss of elasticity, and consequent dilation. Elastin content of the ascending aorta is high and diminishes down to the abdominal.

Thoracic aneurysmp/w - asymptomatic - local compression - descending aorta, back pain localized between the scapulae - dyspnea, stridor, wheezing, or cough - dysphagia - erosion into surrounding structures hemoptysis, hematemesis - spinal cord compression or thrombosis of spinal arteries may result in neurologic symptoms of paraparesis or paraplegia.

Investigation - X ray - CT - MRI

Treatment - Surgical repair of DTA - Endovascular stent grafts

Mural Causes

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Caustic StrictureFollows accidental or suicidal ingestion of strong acids or alkalis. (e.g. caustic soda, ammonia). Often occurs in children.

Acute phase: associated burns of the mouth and pharynx.

Middle and lower eosophagus usually affected as these are sites of temporary hold-up of caustic material where oesophagus is crossed by aortic arch and cardiac sphincter.

Investigation

Eosophageal endoscopy

Barium swallow

Achalasia of the cardiaFailure of relaxation at lower end of oesophagus with progressive dilatation, tortuosity, incoordination of peristalsis and often hypertrophy of the oesophagus above.

A muscle ring at the lower oesophageal sphincter normally relaxes during swallowing. In Achalasia, this muscle ring does not relax as well.

Most common in middle-aged and older adults.

Causes progressive dysphagia over months to years, sometimes with chest-pain.

Complications: Acid reflux, pneumonia, perforation of oesophagus, malignant change in the dilated oesophagus.

InvestigationChest X-Ray- shows the dilated oesophagusOesophageal manometryBarium swallowOesophagoscopy

Reflux OesophagitisProduced by reflux of peptic juice through the incompetent cardiac sphincter into the lower oesophagus.Symptoms: Heartburn, nausea after eating.

Complications: Ulceration Inflammation Stricture formation Barretts oesophagus

Special Investigation: Barium swallow Eosophagogastroduodenoscopy Eosophageal manometry Fibreoptic oesophagoscopy Continuous oesophageal pH monitoring Acid infusion test

Oesophageal CarcinomaUpper two thirds Squamous carcinomas Lower one third Adenocarcinoma

Clinical Features: Dysphagia, enlarged nodes, hoarseness and bovine cough(if present, suggests invasion of left laryngeal nerve by an upper oesophageal tumour)

Risk factors: Squamous carcinoma linked to achalasia and celiac disease. Adenocarcinoma linked to Barretts oesophagus Smoking, alcohol.

Investigation: Barium swallow, Biopsy, Eosophagoscopy, CT scan, Endoscopic ultrasound.

Barretts OesophagusA pre-malignant conditionLong-standing reflux of duodenogastric contents cause metaplasia of the lower oesophageal epithelium to a gastric-type columnar epithelium. Continued inflammation leads to dysplasia and subsequently to malignancy (Adenocarcinoma)

Plummer-Vinson SyndromeA syndrome comprising of dysphagia and iron-deficiency anaemia usually in middle-aged or elderly women.

The dysphagia is associated with hyperkeratinization of the oesophagus and often with the formation of a web in the upper part of the oesophagus.

Premalignant condition, associated with development of carcinoma in the cricopharyngeal region.

Investigation- Oesophagoscopy

Plummer-Vinson Syndrome

Pharyngeal PouchMucosal protrusion between thyropharyngeus and cricopharyngeus muscle (Both forms the inferior constrictor of the pharynx) through Killians dehiscence.An example of pulsion diverticulum, forming as a result of increased intraluminal pressure.Often occurs in men and the elderly.

Symptoms: Dysphagia, regurgitation of food collected in pouch, foetor. Complications: Aspiration pneumonia & lung abscess.

Investigation: Barium swallow- show residual pool of contrast within the pouch.

Congenital atresia

A congenital disorder in which the oesophagus does not develop properly.In most cases, the oesophagus does not connect with the lower oesophagus and stomach.Presence of tracheosophageal fistula (TEF)A surgical emergencySymptoms: Cyanosis/coughing/choking- with attempted feeding. Drooling, Poor Feeding.Complications: Feeding problems, Reflux after surgery, Stricture after surgery due to scarring.

Schatzki RingNarrowing of the lower part of the oesophagus caused by a ring of mucosal tissue or muscular tissue.Subdivided into: A Ring Above oesophagus/stomach junction B Ring At the squamocolumnar junction (lower oesophagus) Complication: Complete obstruction of the eosophagus by a bolus of food which can cause crushing chest pain.Investigation: Barium swallow, oesophagogastroduodenoscopy

Other CausesScleroderma

Diffuse oesophageal spasm

Chagas Disease

Complications of DysphagiaCoughing and choking

Aspiration pneumonia

Malnutrition and dehydration