What is the optimal management of achalasia cardia, including diagnostic steps and definitive therapy options?

Optimal Management of Achalasia Cardia

For achalasia types I and II, laparoscopic Heller myotomy (LHM) with partial fundoplication or pneumatic dilation (PD) are equally effective first-line treatments, while per-oral endoscopic myotomy (POEM) is the definitive preferred treatment for type III (spastic) achalasia. 1, 2, 3

Diagnostic Algorithm

Initial Workup

• Perform upper endoscopy with retroflexed examination of the gastroesophageal junction to exclude pseudoachalasia from occult malignancy or mechanical obstruction 2, 3
• Obtain high-resolution manometry (HRM) as the gold standard to confirm diagnosis and define achalasia subtype (Type I, II, or III) according to Chicago Classification—this subtype determination is critical for treatment selection 1, 2, 3
• Order timed barium esophagram to document degree of esophageal dilation, structural changes (sigmoid deformation), and severity of outflow obstruction 2, 3

Diagnostic Criteria

• Achalasia requires both impaired deglutitive lower esophageal sphincter (LES) relaxation (elevated integrated relaxation pressure) and absent peristalsis 1
• Type I achalasia: negligible esophageal pressurization (classic achalasia) 1
• Type II achalasia: panesophageal pressurization—the most common presenting subtype with best prognosis 1, 3
• Type III achalasia: premature spastic contractions with distal latency <4.5 seconds—obstructive physiology extends beyond the LES into the distal esophagus 1, 4

Treatment Algorithm by Achalasia Subtype

Type I and Type II Achalasia

Primary treatment options (all equally effective):

• Laparoscopic Heller myotomy (LHM) with partial fundoplication: Provides 90% first-year success rates with superior long-term durability; requires 2 days hospitalization with 1-2 week recovery 2, 5

• Pneumatic dilation (PD): Start with 30mm balloon under endoscopic or fluoroscopic guidance, advance to 35mm if symptoms persist at 2-28 days; achieves 90% success at 1 year, 86% at 2 years; anticipate repeat dilations over years; carries ~2% perforation risk 1, 2, 3, 5

• POEM: Comparable efficacy to LHM and PD for types I and II, but requires 20-40 procedures for physician competence and should only be performed in high-volume centers 1, 3

Treatment selection considerations:

• PD has less morbidity and cost compared to LHM, making it preferred for patients seeking outpatient treatment 1
• LHM is preferred when hiatal hernia, significant epiphrenic diverticulum, or need for concurrent anti-reflux surgery exists 1
• POEM offers technical advantages including no abdominal incisions, ability to perform longer myotomies, and avoidance of vagal nerve injury 3

Type III (Spastic) Achalasia

POEM is the definitive preferred treatment because it allows unlimited proximal extension of the myotomy calibrated to the spastic segment visualized on HRM or thickened segment on endoscopic ultrasound, achieving 92% response rates with average myotomy length of 17.2 cm 1, 2, 4

Rationale for POEM superiority:

• Type III achalasia has obstructive physiology extending into the distal esophagus, not just the LES 1, 4
• Standard therapies (PD, LHM) directed only at the LES have consistently worse outcomes in type III disease 1, 4
• POEM allows myotomy extension of 12-16 cm, which is impossible with laparoscopic approaches 4

Alternative if POEM unavailable:

• LHM can be considered but must be extended proximally to address the spastic component, with generally inferior results compared to POEM 4
• PD has limited efficacy and is not recommended as primary therapy for type III achalasia 4

Special Populations and Advanced Disease

End-stage achalasia with sigmoid deformation:

• POEM has a doubling of adverse events in sigmoid esophagus 1, 3
• Esophagectomy should be considered the primary treatment option for sigmoid achalasia with severe dilation and irreversible structural changes 4

EGJ outflow obstruction:

• Many cases resolve spontaneously (12-40% may be early or incomplete achalasia) 1
• Should not justify permanent intervention without comprehensive evaluation and symptom correlation 3
• Functional luminal imaging probe (FLIP) can help clarify diagnosis when equivocal 3

Post-Treatment Management

Reflux Management (Critical Pitfall)

All patients undergoing POEM must receive mandatory proton pump inhibitor (PPI) therapy immediately post-procedure 2, 4

• POEM carries the highest reflux risk: 10-40% rate of symptomatic GERD or ulcerative esophagitis, with 58% showing gastroesophageal reflux on pH-metry 1, 2, 4
• Patients may require indefinite PPI therapy and/or surveillance endoscopy 1, 4
• LHM with partial fundoplication has lower reflux rates compared to POEM 1, 4

Management of Failed Initial Treatment

POEM is superior to pneumatic dilation for patients with failed initial POEM or LHM, with 62% vs 27% success rate in randomized trials 3

Long-Term Surveillance

Patients with achalasia have a 4.6-fold higher risk for esophageal cancer, providing support for endoscopic surveillance 3

Treatments to Avoid

Botulinum toxin injection should not be used as primary therapy as it provides only short-term relief (1-2 years in elderly patients) and requires periodic reinjection with progressively less benefit over time 2, 5, 6

Medical therapy with smooth muscle relaxants (nitrates, calcium channel blockers) is much less effective than invasive procedures, with common side effects and drug tolerance 5

Key Technical Requirements

• POEM requires 20-40 procedures to achieve competence and should only be performed by experienced physicians in high-volume centers 1, 2, 3, 4
• Single-dose antibiotic prophylaxis at time of POEM is sufficient 4
• Myotomy length for POEM must be calibrated to spastic segment on HRM or thickened segment on EUS 4