What are the management options for achalasia cardia?

Management of Achalasia Cardia

Primary Treatment Selection

For type I and II achalasia, pneumatic dilation (PD) and laparoscopic Heller myotomy (LHM) are equally effective with 90% first-year success rates, but for type III (spastic) achalasia, per-oral endoscopic myotomy (POEM) is the definitive preferred treatment due to its ability to perform longer, tailored myotomy. 1, 2

Treatment Algorithm by Achalasia Subtype

Type I (Classic) and Type II (Panesophageal Pressurization) Achalasia

Pneumatic Dilation Protocol:

• Start with 30 mm balloon in first session to minimize perforation risk (0-7%, mortality <1%) 1, 3
• Perform second dilatation 2-28 days later with 35 mm balloon 1, 2
• Consider third session with cautious 40 mm balloon if Eckardt score remains >3 1
• Perform under endoscopic or fluoroscopic control based on local expertise 1
• Effectiveness: 90% at 1 year, 86% at 2 years, with long-term success rates of 97% at 5 years and 93% at 10 years with repeat dilations 1, 2

Predictors of PD Success:

• Post-procedure lower esophageal sphincter pressure (LESP) drop >50% or LESP <10 mmHg predicts good response 4
• Patients over 40 years with pre-treatment LESP ≤32 mmHg and >50% LESP drop achieve better surgical outcomes than PD 4

Laparoscopic Heller Myotomy with Partial Fundoplication:

• Comparable efficacy to PD with 84% success at 3 months and 60% symptom-free at 2 years 4
• Lower reflux rate (4.7%) compared to PD (27.7%) 4
• Higher upfront cost and morbidity but fewer repeat procedures needed 1, 3

Type III (Spastic) Achalasia

POEM is the preferred primary therapy because it allows unlimited proximal extension of myotomy tailored to the extent of esophageal body spasm, with 92% response rates 1, 2, 3

POEM Requirements:

• Must be performed by experienced physicians in high-volume centers (20-40 procedures needed for competence) 1, 2, 3
• Single-dose antibiotic prophylaxis at time of procedure 3
• Mandatory post-procedure proton pump inhibitor therapy due to high reflux risk 1, 3

Stage 4 (End-Stage) Achalasia

Esophagectomy should be considered as primary treatment for severe esophageal dilation (>6-7 cm diameter) with sigmoid deformation, as it addresses irreversible structural changes, prevents ongoing aspiration risk, and eliminates malignancy risk in chronically dilated esophagus 5

• POEM may be attempted only when esophagectomy is not feasible, with significantly tempered expectations 5
• Most published trials for PD, LHM, and POEM specifically excluded end-stage cases 1, 5

Post-Treatment Management

Gastroesophageal Reflux Prophylaxis:

• PPI therapy strongly recommended after all treatments, particularly POEM which has highest reflux risk 1, 3
• PD has 10-40% rate of symptomatic GORD or ulcerative esophagitis 1
• Post-POEM patients may require indefinite PPI therapy and surveillance endoscopy 1, 2, 3

Follow-Up Strategy:

• Up to one-third of PD patients experience symptom recurrence during 4-6 years, but most respond to repeat dilation 1, 2
• Monitor for perforation signs: pain, breathlessness, fever, tachycardia 3
• Water-soluble contrast swallow after dilation can screen for perforation but is not essential 1

Critical Decision Points

Choose PD when:

• Patient prefers less invasive approach with lower upfront morbidity and cost 1, 3
• Patient accepts need for potential repeat procedures over years 1, 2
• Type I or II achalasia with favorable predictors (age <40, LESP characteristics) 4

Choose LHM when:

• Patient over 40 years with LESP ≤32 mmHg and anticipated >50% LESP drop 4
• Patient prioritizes minimizing reflux risk and avoiding repeat procedures 4
• Local surgical expertise available 1

Choose POEM when:

• Type III achalasia (mandatory preferred treatment) 1, 2, 3
• High-volume center with experienced operator available 1, 2, 3
• Patient accepts higher reflux risk and need for indefinite PPI therapy 1, 3

Common Pitfalls

Avoid these errors:

• Performing POEM without adequate operator experience (20-40 cases required) 1, 2, 3
• Failing to subtype achalasia with high-resolution manometry before treatment selection 3, 6
• Neglecting PPI therapy post-procedure, particularly after POEM 1, 3
• Attempting standard treatments for stage 4 achalasia when esophagectomy is indicated 5
• Missing pseudoachalasia from occult malignancy by inadequate retroflexed examination at gastroesophageal junction 3, 5