What are the treatment options for achalasia?

Treatment Options for Achalasia

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

Diagnostic Workup Required Before Treatment Selection

Before initiating any treatment, proper subtyping is essential to optimize outcomes:

• High-resolution manometry (HRM) is the gold standard for diagnosing achalasia and defining the Chicago Classification subtype, which is crucial for phenotype-directed treatment 1
• Esophagogastroduodenoscopy (EGD) with retroflexed examination must be performed to exclude pseudoachalasia from malignancy or other secondary causes 1
• Timed barium esophagram assesses structural changes, confirms outflow obstruction, and evaluates disease severity 1
• Functional luminal imaging probe (FLIP) serves as a useful adjunct when diagnosis is equivocal, measuring impaired esophagogastric junction opening through low distensibility index 1

Treatment Algorithm Based on Achalasia Subtype

Type I and II Achalasia: Three Equivalent Options

All three primary treatments (PD, POEM, LHM) achieve comparable efficacy for types I and II achalasia, with treatment selection based on shared decision-making considering patient preferences and local expertise. 1

Pneumatic Dilation Protocol:

• Start with a 30-mm diameter balloon under endoscopic or fluoroscopic guidance to minimize perforation risk (1.0% vs 3.2% with 35-mm initial dilation) 2
• Schedule a second session 2-28 days later with a 35-mm balloon if symptoms persist, which is safer than initial 35-mm dilation (0.97% vs 9.3% perforation rate) 2
• Consider cautious advancement to 40-mm balloon for persistent symptoms, achieving 90% success rates with graded approach 2
• Expect 90% effectiveness in the first year, with long-term success rates of 97% at 5 years and 93% at 10 years with repeat dilations as needed 3
• Anticipate that up to one-third of patients may experience symptom recurrence during 4-6 years of follow-up, but most can be successfully retreated 3

Laparoscopic Heller Myotomy:

• LHM combined with partial fundoplication provides 90% symptom relief and is the most effective surgical technique 4
• Adding fundoplication significantly reduces postoperative gastroesophageal reflux (8.8% with fundoplication vs 31.5% without; OR 6.3) 4
• Consider LHM when patient prefers definitive single intervention or when local expertise favors surgical approach 1

Per-Oral Endoscopic Myotomy (POEM):

• POEM should only be performed by experienced physicians in high-volume centers, as 20-40 procedures are needed to achieve competence 1
• POEM is comparable in efficacy to LHM for types I and II achalasia, though long-term outcomes data remain limited 1
• Post-POEM patients have higher risk of reflux esophagitis and should be counseled about potential need for indefinite proton pump inhibitor therapy and/or surveillance endoscopy 1

Type III Achalasia: POEM is Preferred

POEM should be considered the preferred primary therapy for type III achalasia because it allows unlimited proximal extension of myotomy to address spastic body contractions, achieving 92% response rates 1, 3

• Type III achalasia is characterized by spastic body contractions requiring myotomy tailored to the proximal extent of esophageal body spasm rather than confined to the lower esophageal sphincter alone 1
• Laparoscopic approaches cannot achieve the same proximal myotomy extension that POEM provides 1
• If POEM expertise is unavailable, LHM remains an acceptable alternative, though outcomes may be inferior 1

Management of Treatment Failure

For patients with failed initial POEM or LHM, repeat POEM is superior to pneumatic dilation (62% vs 27% success rate) 5

Special Populations and Contraindications

Elderly or High Surgical Risk Patients:

• Botulinum toxin injection may be considered specifically for elderly patients or those with high surgical risk who are not candidates for myotomy or pneumatic dilation 6
• Botulinum toxin has modest long-term results compared to other options and frequently requires repeated injections 6

End-Stage Disease:

• Patients with sigmoid esophageal deformation may require esophagectomy rather than sphincter-directed therapy, as POEM has a doubling of adverse events in this population 5

Esophagogastric Junction Outflow Obstruction (EGJOO):

• EGJOO alone should not justify permanent intervention without comprehensive evaluation correlating symptoms with manometric findings 1
• EGJOO can be caused by obesity, hiatal hernia, GERD, external compression, or artifact, requiring exclusion of these alternative diagnoses 1

Critical Post-Treatment Considerations

Gastroesophageal Reflux Management:

• Post-treatment reflux is more common after POEM than after PD or LHM with fundoplication 3
• All post-POEM patients should be counseled about high reflux risk and potential need for indefinite acid suppression 1

Long-Term Surveillance:

• Patients with achalasia have a 4.6-fold higher risk for esophageal cancer, supporting endoscopic surveillance 5

Common Pitfalls to Avoid

• Do not perform initial pneumatic dilation with 35-mm or 40-mm balloons, as this significantly increases perforation risk compared to graded approach starting at 30-mm 2
• Do not perform POEM without adequate training and volume, as 20-40 procedures are required for competence 1
• Do not perform LHM without fundoplication, as this increases postoperative reflux from 8.8% to 31.5% 4
• Do not use botulinum toxin as first-line therapy in young, surgical candidates, as it is reserved for elderly or high-risk patients only 6