Achalasia

Mr Yuen Soon

Laparoscopic Tutor

Consultant Oesophagogastric and Laparoscopic Surgeon

Definition

ach·a·la·sia   (āk'ə-lā'zhə)    n.  The failure of a ring of muscle fibers, such as a sphincter of the esophagus, to relax.

[New Latin : a-1 + Greek khalasis, relaxation (from khalān, to loosen).]

Definition

Achalasia is primary a disorder of motility of the lower

oesophageal or cardiac sphincter. The smooth muscle

layer of the oesophagus has impaired peristalsis and

failure of the sphincter to relax causes a functional

stenosis or functional oesophageal stricture.

Who is this

Who described this?

Who is this?

• First described by Sir Thomas Willis 1672

• Described as Cardiospasm by Von Mikulicz 1881

• Ernest Heller performed the first operation 1913

History

History

• Term Achalasia cioned by Hurt and Rake 1929

• First laparoscopic Hellers performe by Shimi in UK 1991

• Botox introduced 1994

Clinical Features

• 1/100000

• Equal sex distribution

• Occurs at all ages especially after seventh decade

Clinical Symptoms

• Dysphagia

• Regurgitation (80-90%)

• Chest Pain (17-63%)

• Heartburn/Cough/Recurrent Chest Infection/Weight loss

Dysphagia

• Inability to swallow– Non prgressive– Constant

• Due to – motility dysfunction– Cardiac spasm

Regurgitation

• Food refluxing from distal to proximal oesophagus– Usually stale food– Predisposes to

• Halitosis• Chest infections

• Sometimes mistaken for heartburn

Chest pain

• Mechanism unclear– Oesophageal Distention– Oesophageal irritation– Tertiary contraction

• No correlation with manometry

• 84% resolved with Manometry

• Heterogenous cause

Pathophysiology

• Loss of nerve cells in the oesophagus

• Fibrosis and inflammation

• Hypertrophy and degeneration of oesophageal muscle

• Loss of Nitric Oxide deficiency

• Preservation of Acetyl Choline Nerves and other promoters of muscle tone

• Eosinophils

Huh?!?

• What does that all mean

– Oesophageal Motility

– Sphincter dysmotility

• Viral

• Autoimmune

• Allergy

• But truly no one knows

Aetiology

• Barium Swallow

• Endoscopy and Biopsy

• Manometry

Investigations

Barium Swallow

• Characteristic Findings– Aperistalsis of Distal Oesophagus

– Bird Beaking

– Dilatation or tortuousity

GI Motility online (May 2006) | doi:10.1038/gimo53

Figure 1 Esophagrams of a patient with early achalasia pre- and posttreatment.

GI Motility online (May 2006) | doi:10.1038/gimo29

Figure 1 a: Barium esophagram showing a dilated, tortuous esophagus and a ”bird's beak” appearance of the lower esophageal sphincter (LES).

Stages of Achalasia

• 2-3 cm is normal

• 4-5 cm is stage two and bird beak looking

• 5-7 cm is stage three

• 8+ cm is sigmoid or stage 4.

Endoscopy

• To ensure no other causes of symptoms

• Usual findings– Excess stale food in oesophagus– Candidiasis

Manometry

• Characteristic findings– Absence of peristalsis– Pressure maybe hypertonic (Vigourous

Achalasia)– Pressure maybe hypotonic– May have distal barrier function

(Non relaxing sphincter)

GI Motility online (May 2006) | doi:10.1038/gimo22

Figure 2 Esophageal manometric findings in achalasia.

GI Motility online (May 2006) | doi:10.1038/gimo22

Figure 3 Contour plot topographic analysis of esophageal motility in achalasia.

GI Motility online (May 2006) | doi:10.1038/gimo22

Figure 4 Esophageal manometric findings in vigorous achalasia.

GI Motility online (May 2006) | doi:10.1038/gimo22

Figure 5 Esophageal manometric findings in achalasia variant with preserved LES relaxation.

Differential Diagnosis

• Secondary Achalasia– Cancer– Infection– Allergy

• Other Oesophageal Dysmotilities– Diffuse Oesophageal Spasm– Presbyoesophagus– Scleroderma

• Achalasia•   Allgrove's syndrome (AAA syndrome)10, 36•   Hereditary cerebellar ataxia37•   Familial achalasia38•   Sjögren's syndrome39•   Sarcoidosis40•   Postvagotomy41•   Autoimmune polyglandular syndrome type II11• Achalasia with generalized motility disorder•   Multiple endocrine neoplasia (MEN) IIb (Sipple's syndrome)12, 42•   Neurofibromatosis (von Recklinghausen's Disease)13•   Chagas' disease (Trypanosoma cruzi)•   Paraneoplastic syndrome (Anti-Hu antibody)17, 18•   Parkinson's disease8•   Amyloidosis43, 44•   Eosinophilic gastroenteritis45, 46•   Fabry's disease47•   Down syndrome•   Hereditary cerebellar ataxia37•   Achalasia with associated Hirschsprung's disease15•   Hereditary hollow visceral myopathy16• Achalasia associated with cancer. (Cancer-associated achalasia may be due to local invasion of the esophageal myenteric plexus or as a part of a paraneoplastic syndrome.)•   Squamous cell carcinoma of the esophagus•   Adenocarcinoma of the esophagus•   Gastric adenocarcinoma•   Lung carcinoma•   Leiomyoma•   Lymphoma•   Breast adenocarcinoma•   Hepatocellular carcinoma•   Reticulum cell sarcoma•   Lymphangioma•   Metastatic renal cell carcinoma•   Mesothelioma•   Metastatic prostate carcinoma•   Pancreatic adenocarcinoma

Treatment

• Conservative

• Medical– Drugs– Botox– Dilatation

• Surgical– Hellers– Oesophagectomy

Conservative

• Dietetic Support

• Enteral forms of feeding

• Stent

Medical

• Drugs– Seldom long lasting– Seldom effective

– Nitrates (GTN)– Calcium Channel Antagonist (Nifedipine)– Sildenafil (Viagra)

Medical

• Dilatation– 60% success at a year and 24% at 5 years

following single dilatation– Symptoms reoccur in 50% within 5 years– In general 60% have good results at 5 years

with one or more dilatation

Medical

• Predictors of good outcome– Low residual pressure– Older patients

• Complications – Perforation 3-7% (0-21% Range)– Reflux 2%– Higher rate of complication if followed by

myotomy

Medical

• Botox – High quality symptom relief

• 1 month 75-100%• 6 month 44-100%

– Duration of response upto 15 months– 50% will need other forms of treatment within

2 years– Reduces Sphincteric pressure by 40%

Botox

• Increases operative complications

• Recommended only for– Elderly– Low pressure sphincter

Dilatation vs Botox

Author Annese89 Vaezi86 Mikaeli36

* Ghoshal87 Muehldorfer88

  BoT PD BoT PD BoT PD BoT PD BoT PD*These authors used 200 U of Dysport.NR: not reported; LESP: lower esophageal sphincter pressure.Modified from Zhao and Pasricha.67

Response rate

1 month 8/8 8/8 14/20 15/20 13/20 18/20 6/8 8/10 9/12 10/12

12 months

6/8 NR 7/20 14/20 3/20 10/19 3/8 6/8 NR NR

Reduction in LESP

1 month -49% -72% -6 % -66% -24% -26% -53% -62% -44% -51%

12 months

NR NR NR NR NR NR NR NR NR NR

Reduction in retention

1 month 47% 59% 33.6% 50.2% NR NR NR NR NR NR

12 months

NR NR 13.4% 54.6% NR   NR NR NR NR

Surgery

• Laparoscopic

• Long myotomy 6-8cm above and 3 below

• Good long term results for dysphagia 90-95%

Surgery

• Reflux 17-28% to 6% if antireflux procedure added

• Oesophageal perforations 1-5%

• Pneumothorax 3%

Medical vs Surgical  Dilation (n = 32) Surgery (n = 42)

LESP, lower esophageal sphincter pressure.

Source: Modified from Csendes et al.34

Type of procedure Mosher dilation (12–15 psi for 10–20 seconds) Myotomy + antireflux (Dor)

Clinical response 65% 95%LESP at 5 years (% baseline)

16 mm Hg (50%) 10 mm Hg (25%)

GE junction at late follow-up (% baseline) 7.2 mm (270%) 9 mm (321%)

Esophageal diameter at late follow-up (% base line) 29 mm (70%) 26 mm (50%)

Reflux 8% 28%

Perforation 5.60% —

Deaths 0 0

Need for surgery 22% —

Surveillance

• Do we need it

• Rise in Squamous cancers of oesophagus– 33-100x

• ie 3.4/1000 patient years vs 0.1/1000 patient years

– If done then needs chromoendoscopy from 10 years after symptoms starts

Questions

Other Dysmotilities

Motor disorder Manometric abnormality

Achalasia

Absent, incomplete, or abnormally timed LES relaxation Absent peristalsis May have elevated resting LES and intraesophageal pressures

Diffuse esophageal spasm

>20% of swallows result in simultaneous contractions May have multipeaked and/or prolonged contractions Amplitude of the contractions may be increased, normal, or decreased

Hypertensive peristalsis (nutcracker esophagus)Peristaltic contractions of increased amplitude (>180 mmHg) and/or increased duration (>8 sec)

Hypertensive LESIncreased resting LES pressure (>40 mm Hg above intragastric pressure)

Hypotensive peristaltic contractions (ineffective peristalsis)

Decreased amplitude (<30 mmHg) peristaltic or nonperistaltic contractions in distal esophagus with 30% of wet swallows With or without hypotensive LES

Hypotensive LES and increased frequency of TLESRLES pressure <10 mmHg TLESRs cannot be evaluated by usual manometric studies