What are the symptoms and treatment options for achalasia?

Achalasia: Symptoms and Treatment

Clinical Presentation

Achalasia typically presents with progressive dysphagia to both solids and liquids, regurgitation of undigested food, chest pain, and weight loss. 1, 2 Patients may also experience respiratory symptoms from aspiration of retained esophageal contents. 2, 3

Key Symptoms to Identify:

• Dysphagia: Progressive difficulty swallowing both solids and liquids (distinguishes from mechanical obstruction) 1, 2
• Regurgitation: Undigested food, often worse when lying down 1, 3
• Chest pain: Present in many patients, can mimic cardiac pain 1, 2
• Weight loss: Results from progressive difficulty with oral intake 1, 2
• Respiratory symptoms: Aspiration, chronic cough, recurrent pneumonia 2, 3

Diagnostic Evaluation

All patients suspected of having achalasia must undergo comprehensive evaluation including upper endoscopy with careful retroflexed examination, timed barium esophagram, and high-resolution manometry (HRM) to confirm diagnosis and define subtype. 4, 5

Diagnostic Algorithm:

1. Upper endoscopy (EGD): Identify frothy retained secretions, puckered gastroesophageal junction, and exclude pseudoachalasia from malignancy with retroflexed examination 5, 4
2. Timed barium esophagram: Confirms outflow obstruction, demonstrates structural changes (bird's beak appearance), and can reveal subtle narrowing; 13-mm barium tablet administration provides additional evidence 5, 4
3. High-resolution manometry (HRM): Gold standard for diagnosis and crucial for defining Chicago Classification subtype (Types I, II, or III), which has critical therapeutic implications 5, 4, 6
4. Functional luminal impedance planimetry (FLIP): Useful adjunct when diagnosis is equivocal; impaired EGJ opening with low distensibility index supports achalasia diagnosis 5, 4

Treatment Options by Achalasia Subtype

Type I and Type II Achalasia

For Type I and Type II achalasia, pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), and per-oral endoscopic myotomy (POEM) are all effective first-line therapies; the decision should be based on shared decision-making considering patient characteristics, preferences, and local expertise. 5, 4

Treatment Selection Framework:

• Pneumatic dilation (PD): Less morbidity and cost compared to surgical options; provides good relief in 85-93% of cases but anticipate repeat dilations over years 5, 4

  • Perforation risk 0-7% (mostly 3-4%) with mortality <1% 4
  • Most cost-effective alternative but less long-term efficacy than myotomy 7

• Laparoscopic Heller myotomy (LHM): Highly efficacious in randomized controlled trials; provides best long-term symptom control 5, 7

  • Typically performed with partial fundoplication (Toupet or Dor) to prevent reflux 5
  • Established long-term efficacy data 8

• POEM: Superior to PD and noninferior to LHM in multicenter randomized controlled trials; clinical success rates 89-97% 5, 8

  • Critical caveat: Post-POEM reflux is significant concern with esophagitis in 19-33% and abnormal pH studies in 47.8% of patients 8
  • Patients must be advised about potential indefinite proton pump inhibitor therapy and/or surveillance endoscopy before proceeding 5, 8, 4
  • Should only be performed by experienced physicians in high-volume centers (20-40 procedures needed for competence) 5, 4
  • Insufficient data for advanced esophageal dilation, sigmoidization, epiphrenic diverticulum, and hiatal hernia 5, 8

Type III Achalasia (Spastic Achalasia)

POEM should be considered the preferred treatment for Type III achalasia because it allows unlimited proximal extension of myotomy tailored to the extent of esophageal body spasm, which is critical for optimal outcomes in this subtype. 5, 4

• Type III achalasia is characterized by spastic body contractions with distal latency <4.5 seconds and has the poorest response to all treatments 6, 9
• Symptoms are best palliated with myotomy calibrated to the proximal extent of esophageal body spasm rather than confined to LES alone 5
• POEM provides advantage of unlimited proximal extension versus laparoscopic approach, though long-term outcomes on "long myotomies" are limited 5

Post-Treatment Management

Immediate Post-Procedure Care:

• Pharmacologic acid suppression: Strongly consider in immediate post-POEM setting given increased reflux risk 4
• Single dose of antibiotics: May be sufficient for antibiotic prophylaxis at time of POEM 4
• Monitor for perforation: Suspect if patients develop pain, breathlessness, fever, or tachycardia after any intervention 4

Long-Term Surveillance:

• Post-POEM patients: High risk for reflux esophagitis; require potential indefinite PPI therapy and/or surveillance endoscopy 5, 8
• Monitor for complications: Pulmonary aspiration, chest infections, persistent dysphagia, and weight loss are most significant complications affecting morbidity and mortality 4

Special Considerations

Esophagogastric Junction Outflow Obstruction (EGJOO):

Patients with EGJOO alone and/or nonachalasia spastic disorders on manometry should undergo comprehensive evaluation with symptom correlation; POEM should only be considered case-by-case after other less invasive approaches are exhausted. 5

• EGJOO is not pathognomonic for any diagnosis and should not, in isolation, justify permanent intervention 5
• Many cases resolve spontaneously (20-40%), but 12-40% end up being treated as achalasia 5
• Always image the EGJ (EUS, CT) to rule out obstruction from alternative causes (obesity, hiatal hernia, external compression, malignancy) 5

Anticoagulation Management:

• Low-risk patients: Discontinue anticoagulants prior to dilation with preprocedure prothrombin time 4
• High-risk patients: Discontinue oral anticoagulants and transition to IV heparin; stop 4-6 hours before and resume 4-6 hours after procedure 4