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 aetiology behind inflammation
Inflammatory destruction of inhibitory nitrinergic neurons in the oesophageal myenteric (auerbach) plexus -> loss of peristalsis and lower oesophageal sphincter relaxation
Possible trigger factors:
- Infection
- Autoimmunity
- Genetic factors
- Triple-A (Allgrove) syndrome

Question 3

Risk factors for achalasia

Answer 3

Triple A (Allgrove) syndrome
Herpes and measles virus
Autoimmune disease – Abs against myenteric plexus
HLA class II antigens
Consanguineous parents (incest)

Question 4

Symptoms of achalasia

Answer 4

Dysphagia
- Posture changes to aid swallowing
- Solids AND liquids not tolerated
Retrosternal pressure or pain
Regurgitation
Weight loss
Heartburn
Slow eating
Aspiration
Recurrent chest infection
Globus sensation
Hiccups

Question 5

Investigations for achalasia

Answer 5

CXR: widened mediastinum | double right heart border | air fluid level | absence of normal gastric air bubble
Barium swallow: dilated oesophagus that tapers to the sphincter (bird beak sign)
OGD + biopsy: often normal, exclude malignancy
Manometry: Elevated resting lower sphincter pressure, incomplete relaxation with wet swallows

Question 6

Management for achalasia

Answer 6

symptomatic
Initial treatment will depend on whether the patient is a surgical candidate
All patients considered for pneumatic dilatation should be fit enough to undergo surgery

Medical:
Calcium channel blockers e.g. nifedipine or verapamil (Interim while waiting for definitive treatment OR first line for non-surgery candidates)
GTN spray
Pneumatic dilatation
- Outpatient, under sedation
- Air-inflated balloons used to apply mechanical stretch to the lower oesophageal sphincter to tear its muscle fibres
Botulinum toxin injection

Surgical:
Laparoscopic cardiomyotomy
Peroral endoscopic myotomy (POEM)
Gastrostomy or oesophagectomy (For frail and older patients)
Oesophagectomy

Question 7

Complications of achalasia

Answer 7

Aspiration pneumonia
GORD
Oesophageal carcinoma

Question 8

Prognosis for achalasia

Answer 8

No known cure
Treatment is symptomatic
Health-related quality of life usually remains reduced, with a degree of persistent dysphagia, chest pain, or reflux symptoms