Achalasia

Question 1

What is it?

Answer 1

Motility disorder: coordinated peristalsis is lost and the lower oesophageal sphincter fails to relax

Question 2

Why does it happen?

Answer 2

Degeneration of the myenteric plexus in the distal oesophagus and LOS

Question 3

What does it result in?

Answer 3

Distal obstruction of the oesophagus

Question 4

What is the presentation?

Answer 4

Dysphagia
Weight loss
Chest pain
Regurgitation and chest infection

Question 5

What is the differential diagnosis of dysphagia?

Answer 5

Benign stricture
Malignant stricture (oesophageal cancer)
Motility disorders (e.g. achalasia)
Eosinophilic oesophagitis
Extrinsic compression (e.g. lung cancer)

Question 6

How is diagnosed?

Answer 6

Characteristic findings on contrast swallow (barium swallow) showing dilated tapering oesophagus
Manometry (usually after endoscopy) shows high pressure in the lower oesophageal sphincter at rest (usually above 45mmHg, normal being 10mmHg): cardinal feature is failure of the lower oesophageal sphincter to relax after swallowing and absence of useful peristaltic contraction in the lower oesophagus

Question 7

What are the treatment options?

Answer 7

Pharmacological: calcium channel blockers and nitrated may help relax the sphincter
Endoscopic: pneumatic balloon dilation, botulinum toxin injection (repeat every few months)
Surgical: Heller’s cardiomyotomy (then PPIs)

Question 8

What are the complications?

Answer 8

Aspiration pneumonia

Increased risk of squamous cell oesophageal carcinoma

Question 9

What is the onset?

Answer 9

Usually in 3rd - 5th decade of life