Achalasia

Question 1

Define achalasia

Answer 1

Oesophageal motility/Motor disorder characterised by oesophageal aperistalsis and insufficient lower oesophageal sphincter relaxation in response to swallowing

Question 2

Aetiology of achalasia

Answer 2

Unknown

Inflammatory destruction of inhibitory nitrinergic neurones in the oesophageal myenteric plexus (Auerbach) -> loss of peristalsis and lower oesophageal sphincter relaxation

Question 3

What are the possible triggers for achalasia

Answer 3

Infection e.g. Herpes, Measles
Autoimmunity I.e. Abs against the myenteric plexus
Genetic factors e.g. HLA class II antigens
Triple-A (Allgrove) syndrome
Incest

Question 4

Epidemiology of achalasia

Answer 4

Incidence increases with age
Median age of diagnosis is 53
Increasing incidence and prevalence

Question 5

Symptoms of achalasia

Answer 5

Dysphasia (posture changes to aid swallowing, solids AND liquids no tolerated)
Retrosternal pressure or pain
Regurgitation 
Weight loss
Heartburn 
Slow eating
Aspiration 
Recurrence chest infections
Globes sensation
HIccups

Question 6

Signs of achalasia on examination

Answer 6

Aspiration pneumonia’s
Malnutrition and weight loss
Oesophageal malignancy (15x risk)

Question 7

Investigations for achalasia

Answer 7

CXR: widened mediastinum | double right heart border | air-fluid level in upper chest | absence of normal gastric air bubble

Barium swallow: Dilate oesophagus that tapers into the sphincter - BIRD BEAK appearance (normal in early disease)

OGD + Biopsy: Often normal | constricted lower sphincter | mucosa obscured by retained saliva with frothy appearance
Advanced = dilate and tortuous + food debris

Manometers: elevated resting lower oesophageal sphincter pressure >45mmHg | incomplete lower oesophageal sphincter (LOS) reaction with wet swallows
Absence of peristalsis in the distal oesophagus