Achalasia Type 3

Achalasia type III (spastic achalasia) is the manometric subtype with impaired lower oesophageal sphincter relaxation and premature (spastic) contractions of the distal oesophagus in at least 20% of swallows.

Chicago classification: integrated relaxation pressure over 15 mmHg, no normal peristalsis, premature contractions (distal latency under 4.5 seconds) in 20% or more of swallows.

Least common subtype (about 5 to 15%), associated with opioid use, older age, diabetes, and sometimes neuronal degeneration of inhibitory neurons.

Prognosis is worst among the subtypes with standard therapy; vagal and myenteric involvement.

Distinguish from distal oesophageal spasm (normal relaxation).

• Severe chest pain (most frequent and severe), dysphagia, regurgitation, weight loss.

• Pain often prominent and unresponsive to nitrates and calcium channel blockers.

Differential diagnosis: opioid induced oesophageal dysfunction, cardiac disease, eosinophilic oesophagitis.

• Exclude cardiac disease first.
• High resolution manometry confirms premature contractions with raised integrated relaxation pressure.
• Barium oesophagram: corkscrew oesophagus, bird beak, spasm.
• Upper endoscopy with biopsies; endoscopic ultrasound or CT for pseudoachalasia; stop opioids and repeat manometry.
• EndoFLIP: repetitive retrograde contractions.
• Chagas serology in endemic exposure.

• POEM with a long myotomy extending proximally across the spastic segment (guided by manometry, often 12 to 18 cm) is the preferred treatment: success 85 to 90% in type III, superior to pneumatic dilation (about 40 to 50%).
• Laparoscopic Heller myotomy with extended proximal myotomy (thoracoscopic or laparoscopic) is an alternative with lower success than in other subtypes.
• Botulinum toxin 100 to 200 units injected in the spastic segment and lower oesophageal sphincter for frail patients; effect 3 to 6 months.
• Medical therapy for pain: diltiazem 60 to 120 mg three times daily, nitrates, sildenafil 25 to 50 mg, tricyclics (amitriptyline 10 to 50 mg), peppermint oil.
• Stop opioids if present.
• PPI after POEM (omeprazole 20 to 40 mg daily) for reflux.
• Follow up with Eckardt score and timed barium oesophagram; manage persistent pain with neuromodulators.
• Surgical oesophagectomy for refractory end stage disease.