What is the workup and treatment of achalasia?

Workup and Treatment of Achalasia

Diagnosis and Workup

High-resolution manometry (HRM) is the gold standard for diagnosis and subtyping of achalasia, which is essential for guiding appropriate treatment selection. 1

A comprehensive diagnostic evaluation should include:

1. High-resolution manometry (HRM) - Confirms diagnosis and determines achalasia subtype (I, II, or III)
2. Upper endoscopy - Rules out pseudoachalasia (e.g., malignancy at gastroesophageal junction)
3. Timed barium esophagram - Demonstrates characteristic "bird's beak" appearance and assesses esophageal emptying
4. Functional luminal impedance planimetry (FLIP) - Provides additional assessment of esophagogastric junction distensibility

Treatment Approach

Treatment selection should be based on achalasia subtype, available expertise, presence of hiatal hernia, and patient-specific factors:

First-Line Treatment Options

1. Per-Oral Endoscopic Myotomy (POEM)

   • Preferred treatment for type III achalasia with 92% response rate 1
   • Comparable success to LHM for types I and II
   • Advantages: minimally invasive, no abdominal incisions, rapid recovery
   • Disadvantages: higher risk of post-procedure reflux requiring PPI therapy
   • Technique: submucosal tunnel 10-15cm proximal to LES, extending 2-4cm onto gastric cardia, with circular muscle myotomy

2. Laparoscopic Heller Myotomy (LHM) with Partial Fundoplication

   • Success rate of 84-94% 1
   • Advantages: lower post-procedure reflux rates
   • Disadvantages: more invasive, requires general anesthesia, limited ability to extend myotomy proximally
   • Should be combined with partial fundoplication (Toupet or Dor) to prevent reflux

3. Pneumatic Dilation (PD)

   • Effective in 90% of patients in first year, with long-term success rates of 97% at 5 years with repeat dilations 1
   • Technique: performed under endoscopic or fluoroscopic control, balloon positioned at esophagogastric junction, inflated for 1-3 minutes
   • Start with 30mm balloon, consider subsequent dilations with larger balloons if symptoms persist

Alternative/Second-Line Options

1. Botulinum Toxin Injection

   • Reserved for patients who cannot undergo balloon dilation and are not surgical candidates 2
   • Limited efficacy and durability compared to other treatments

2. Pharmacological Therapy (Calcium Channel Blockers, Nitrates)

   • Only used in patients who are not candidates for other interventions 2
   • Poor long-term efficacy due to tolerance and side effects 3

Treatment Algorithm Based on Achalasia Subtype

1. Type I and II Achalasia:

   • POEM, LHM, and PD are all effective options
   • Consider patient preference regarding invasiveness and reflux risk

2. Type III Achalasia:

   • POEM is clearly superior due to ability to perform longer myotomy extending into esophageal body 1

3. End-Stage Achalasia/Sigmoid Esophagus:

   • POEM has been used successfully but adverse events may double 1
   • Consider referral to high-volume centers with expertise

Post-Procedure Care and Complications

1. Reflux Management:

   • All treatments can cause reflux, with POEM having highest risk
   • Proton pump inhibitor therapy and surveillance endoscopy recommended

2. Monitoring for Complications:

   • Monitor for at least 2 hours post-procedure
   • Watch for signs of perforation: pain, breathlessness, fever, tachycardia
   • Provide clear diet instructions and contact information for on-call team

3. Recurrent Dysphagia:

   • May require repeat intervention
   • POEM shows higher success rates compared to repeat pneumatic dilation 1

Important Considerations

• POEM and LHM should be performed by experienced physicians in high-volume centers (20-40 procedures needed for competence) 1
• Laparoscopic myotomy with fundoplication is considered the most effective surgical technique with best long-term symptom control 4, 3
• Pneumatic dilation is more cost-effective but has lower long-term efficacy than surgical myotomy 3
• The field is evolving with newer interventions such as stenting and cell transplant under active investigation 5