What are the treatment options for achalasia?

Treatment for Achalasia

For achalasia types I and II, pneumatic dilation (PD), per-oral endoscopic myotomy (POEM), and laparoscopic Heller myotomy (LHM) are all effective primary treatments with comparable outcomes, while POEM is the preferred treatment for type III achalasia due to its ability to perform longer myotomies extending beyond the lower esophageal sphincter. 1

Diagnostic Workup Before Treatment

Before initiating therapy, confirm the diagnosis and achalasia subtype through:

• Esophagogastroduodenoscopy (EGD) with careful retroflexed examination to exclude pseudoachalasia, looking for frothy retained secretions and puckered gastroesophageal junction 1
• High-resolution manometry (HRM) as the gold standard to define achalasia subtype according to Chicago Classification, which is crucial for phenotype-directed treatment 1
• Timed barium esophagram to assess structural changes, confirm outflow obstruction, and evaluate disease severity 1
• Functional luminal imaging probe (FLIP) as an adjunct when diagnosis is equivocal, assessing impaired EGJ opening through low distensibility index 1

Treatment Selection Algorithm by Achalasia Subtype

Type I Achalasia (Classic - No Pressurization)

All three primary treatments (PD, POEM, LHM) are equally effective. 1 Choose based on:

• Pneumatic dilation: Start with 30mm balloon, progress to 35mm at 2-28 days, with 90% effectiveness in first year and 93% success at 10 years with repeat dilatations 2
• POEM or LHM: Consider for younger patients or those preferring definitive single intervention 1

Type II Achalasia (With Panesophageal Pressurization)

Best outcomes with any of the three primary treatments. 1, 2 This subtype has the most favorable prognosis regardless of treatment modality selected 1

Type III Achalasia (Spastic)

POEM is the preferred treatment because it allows unlimited proximal extension of myotomy (averaging 17.2 cm) to address spastic contractions throughout the smooth muscle esophagus, achieving 92% response rates. 1 LHM and PD have inferior outcomes for this subtype because they only address the LES 1

Pneumatic Dilation Technical Details

• Initial session: Use 30mm diameter balloon under endoscopic or fluoroscopic guidance to minimize perforation risk 2
• Second session: Advance to 35mm balloon at 2-28 days if symptoms persist 2
• Third session: Cautiously consider 40mm balloon only if needed 2
• Long-term outcomes: 97% success at 5 years and 93% at 10 years with repeat dilatations as needed 2
• Recurrence management: Up to one-third experience symptom recurrence during 4-6 years, but most respond successfully to repeat dilation 2

POEM Technical Considerations

POEM requires 20-40 procedures to achieve competence and should only be performed by experienced physicians in high-volume centers. 1, 2

Technical advantages over LHM:

• No abdominal incisions with more rapid recovery 1
• Ability to perform longer myotomies without mediastinal dissection 1
• Avoidance of vagal nerve injury 1
• No intra-abdominal adhesions that might hinder future surgery 1
• Option to avoid general anesthesia with airway intubation 1

Critical post-POEM management:

• Pharmacologic acid suppression should be strongly considered immediately post-POEM given increased reflux risk, with abnormal acid exposure occurring in 41-56% and esophagitis in 41-65% of patients 1
• Follow-up endoscopy and/or pH monitoring at 6-12 months for objective evaluation of acid exposure 1
• Indefinite proton pump inhibitor therapy may be required, taken 30-60 minutes before meals for adequate absorption 1

Laparoscopic Heller Myotomy

LHM combined with partial fundoplication (Toupet or Dor) provides 90% symptom relief with lower post-procedure reflux rates (8.8% with fundoplication vs 31.5% without). 3

Key technical points:

• Requires surgical isolation of EGJ with division of phrenoesophageal ligament and short gastric vessels 1
• Fundoplication is essential to prevent pathologic reflux, reducing incidence from 31.5% to 8.8% 3
• Potential for fundoplication-related obstructive dysphagia in aperistaltic esophagus 1

Management of Failed Initial Myotomy

POEM is superior to pneumatic dilation for patients with failed initial POEM or LHM (62% vs 27% success rate in randomized trial), with no significant difference in esophagitis, reflux symptoms, or serious adverse events. 1

Before retreatment, perform comprehensive evaluation including:

• Repeat esophagogastroduodenoscopy 1
• High-resolution manometry 1
• Timed barium esophagram 1

Special Populations and Contraindications

Esophagogastric Junction Outflow Obstruction (EGJOO)

EGJOO alone should not justify permanent intervention. 1 This manometric finding requires:

• Comprehensive evaluation with endoscopic ultrasound and CT to exclude pseudoachalasia, infiltrative disease, cancer, or mechanical obstruction 1
• Correlation with symptoms before considering treatment 1
• POEM only on case-by-case basis after exhausting less invasive approaches 1
• Recognition that 20-40% resolve spontaneously 1

End-Stage Achalasia and Sigmoid Esophagus

Patients with sigmoid deformation have doubling of adverse events with POEM and may require esophagectomy rather than sphincter-directed therapy. 1

Long-Term Surveillance

Patients with achalasia have 4.6-fold higher risk (95% CI, 2.3-9.2) for esophageal cancer, providing support for endoscopic surveillance, though not currently routinely implemented. 1

Common Pitfalls

• Do not rely on reflux symptoms alone post-POEM - objective pH testing is essential as symptoms may be from nonreflux esophageal acidification due to food stasis and acid fermentation rather than true GERD 1
• Avoid POEM in patients with prior anti-reflux or bariatric surgery without careful evaluation, as similar manometric findings can occur post-operatively 1
• Do not perform short myotomy for type III achalasia - extend myotomy to proximal extent of spasm, which POEM uniquely allows 1