Achalasia

Question 1

Definition

Answer 1

A condition in which the normal muscular activity of the oesophagus is disturbed (absent or uncoordinated) due to FAILURE OR INCOMPLETE RELAXATION OF THE LOWER OESOPHAGEAL SPHINCTER.

This leads to delay in the passage of swallowed material into the stomach

Question 2

Aetiology

Answer 2

it is caused by degeneration of the ganglion cells of the myenteric plexus in the oesophagus due to an unknown cause

· NOTE: oesophageal infection with Trypanosoma cruzi seen in Central/South America produces a similar disorder (CHAGAS DISEASE)

Question 3

Epidemiology

Answer 3

· It may occur at any age (mainly 25-60 yrs)

· Affects both sexes equally

· Annual incidence 1/100,000

Question 4

Presenting symptoms

Answer 4

INSIDIOUS onset and gradual progression of:

· Intermittent dysphagia involving solids and liquids
· Difficulty belching
· Regurgitation (particularly at night)
· Heartburn
· Chest pain (atypical/cramping, retrosternal)
· Weight loss (because they are eating less)

Question 5

Signs on physical examination

Answer 5

· May show signs of complications:

o Aspiration pneumonia
o Malnutrition
o Weight loss

Question 6

Investigations (CXR)

Answer 6

o Widened mediastinum

o Double right heart border (dilated oesophagus)

o Air-fluid level in the upper chest

o Absence of the normal gastric air bubble

Question 7

Investigations (other)

Answer 7

· Barium swallow may show:
o Dilated oesophagus which smoothly tapers down to the sphincter (beak-shaped)

· Endoscopy to exclude malignancy (which could mimic achalasia)

· Manometry (used to assess pressure at the LOS) may show:
o Elevated resting LOS pressure (> 45 mm Hg)
o Incomplete LOS relaxation
o Absence of peristalsis in the smooth muscle portion of the oesophagus

· NOTE: you may do serology for antibodies against T. cruzi if CHAGAS DISEASE is a possibility (and blood film may detect parasites)