What is the best management approach for achalasia?

Management of Achalasia

For type I and type II achalasia, choose between pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), or per-oral endoscopic myotomy (POEM) based on shared decision-making; for type III achalasia, POEM is the definitive preferred treatment. 1

Diagnostic Workup Required Before Treatment

Before any intervention, confirm the diagnosis and subtype with:

• Upper endoscopy (EGD) with careful retroflexed examination to identify retained secretions, puckered gastroesophageal junction, and exclude pseudoachalasia from malignancy 1, 2
• High-resolution manometry (HRM) as the gold standard to confirm diagnosis and define Chicago Classification subtype—this is crucial as treatment outcomes vary significantly by subtype 1
• Timed barium esophagram to confirm outflow obstruction, assess structural changes, and establish baseline severity; consider 13-mm barium tablet administration for subtle narrowing 1, 2
• Functional luminal impedance planimetry (FLIP) as an adjunct when diagnosis is equivocal on HRM, looking for low distensibility index 1

Treatment Algorithm by Achalasia Subtype

Type I and Type II Achalasia

All three major interventions (PD, LHM, POEM) are equally effective with >90% symptom improvement rates. 1 The decision should be based on:

Choose Pneumatic Dilation when:

• Patient prefers outpatient procedure with lower upfront cost and morbidity 1, 2
• Patient accepts need for repeat dilations over years (86% success at 2 years, 93% at 10 years with repeat procedures) 2
• Start with 30mm balloon to minimize perforation risk (2% perforation rate overall) 3, 2
• Rare secondary severe GERD 3

Choose Laparoscopic Heller Myotomy when:

• Patient prefers single definitive procedure with lower need for reintervention 2
• Myotomy should extend 2-3 cm onto stomach and be combined with partial fundoplication to prevent severe GERD and peptic stricture 3, 4
• Requires 1-2 days hospitalization with 1-2 week recovery 3

Choose POEM when:

• High-volume center with experienced operator available (20-40 procedures needed for competency) 1, 5
• POEM has been found superior to PD and noninferior to LHM in multicenter RCTs 1
• Patient accepts highest reflux risk (58% show gastroesophageal reflux on pH-metry) and need for indefinite PPI therapy 1, 5, 2

Type III Achalasia (Spastic)

POEM is the definitive preferred treatment for type III achalasia. 1, 5 This is non-negotiable when expertise is available because:

• Type III requires myotomy tailored to the proximal extent of esophageal body spasm, not just the LES 1
• POEM allows unlimited proximal extension of myotomy (averaging 17.2 cm), which laparoscopic approaches cannot achieve 1, 5
• Meta-analyses show 92% response rate in type III achalasia with POEM 1, 5
• Standard therapies limited to the LES have consistently worse outcomes in type III 1, 5

If POEM unavailable:

• LHM can be considered but must be extended proximally with generally inferior results 5
• PD has limited efficacy and is not recommended as primary therapy for type III 5

Post-Treatment Management

Acid suppression is mandatory after all treatments, especially POEM:

• Single dose antibiotics at time of POEM may be sufficient for prophylaxis 2
• Pharmacologic acid suppression strongly recommended immediately post-POEM 2
• Post-POEM patients require indefinite PPI therapy and surveillance endoscopy given 58% reflux rate on pH-metry 1, 5, 2
• 10-40% rate of symptomatic GERD or ulcerative esophagitis across all treatments 2

Post-procedure monitoring:

• Obtain esophagram to rule out leak based on clinical suspicion or local practice 1
• Suspect perforation if patient develops pain, breathlessness, fever, or tachycardia after any intervention 2

Special Considerations and Pitfalls

Esophagogastric Junction Outflow Obstruction (EGJOO):

• EGJOO alone is not pathognomonic for achalasia and should not justify permanent intervention in isolation 1
• Many cases resolve spontaneously 1
• Image the EGJ with EUS or CT to rule out mechanical obstruction, hiatal hernia, external compression, or submucosal masses 1
• POEM for EGJOO should only be considered case-by-case after less invasive approaches exhausted 1

Advanced/End-Stage Disease:

• Insufficient data on POEM efficacy for advanced esophageal dilation, sigmoidization, epiphrenic diverticulum, and hiatal hernia 1
• Sigmoid achalasia has doubled adverse events with POEM 1
• Esophagectomy should be considered primary treatment for sigmoid achalasia with severe dilation as it addresses irreversible structural changes 5

Anticoagulation Management:

• Low-risk patients: discontinue anticoagulants with preprocedure prothrombin time 2
• High-risk patients: transition to IV heparin, stop 4-6 hours before, resume 4-6 hours after procedure 2

Treatments to Avoid as Primary Therapy

Medical therapy is much less effective than invasive procedures:

• Calcium channel blockers and nitrates have variable results with common side effects and drug tolerance 3, 6, 7
• Botulinum toxin injection (100-200 units) provides only short-term relief, most effective in elderly with 1-2 year symptom relief 3, 6, 7
• Reserve botulinum toxin only for patients who cannot undergo PD and are not surgical candidates 6, 7