What are the management options for achalasia in children?

Management of Achalasia in Children

Primary Treatment Recommendation

Laparoscopic Heller myotomy (LHM) with partial fundoplication should be the initial definitive treatment for most children with achalasia, as it provides superior long-term symptom control compared to pneumatic dilation, which has lower success rates in pediatric populations. 1, 2, 3

Diagnostic Workup

Before initiating treatment, establish the diagnosis and achalasia subtype through:

• Upper endoscopy with retroflexed examination of the gastroesophageal junction to exclude pseudoachalasia from occult malignancy 4
• High-resolution manometry (HRM) to confirm diagnosis and define achalasia subtype (Type I, II, or III) according to Chicago Classification, which has critical therapeutic implications 4
• Timed barium esophagram to document degree of esophageal dilation, structural changes, and outflow obstruction 4
• Look specifically for: dysphagia duration, regurgitation patterns, chest pain, weight loss trajectory, and aspiration symptoms 4, 2

Treatment Algorithm by Achalasia Subtype

Type I and Type II Achalasia

First-line: Laparoscopic Heller myotomy with partial fundoplication

• Provides 90% first-year success rates with superior long-term durability 5, 4
• Lower reflux rates compared to POEM due to concomitant fundoplication 5
• Pediatric studies show 65% of children initially treated with myotomy required further interventions, but this remains better than pneumatic dilation outcomes 2

Alternative: Pneumatic dilation

• Consider when surgical expertise unavailable or family preference for less invasive approach 3
• Use graduated protocol starting with 30mm balloon to minimize perforation risk 4
• Critical caveat: Pediatric data suggest lower success rates than in adults, with 85% of children initially treated with botulinum toxin ultimately requiring myotomy 2
• Anticipate repeat dilations over years (90% success at 1 year, 86% at 2 years) 4
• Perforation risk 0-7% with mortality <1% 4

Type III (Spastic) Achalasia

Definitive preferred treatment: POEM (Per-Oral Endoscopic Myotomy)

• Allows unlimited proximal extension of myotomy calibrated to the spastic segment visualized on HRM or thickened segment on endoscopic ultrasound 5, 4, 6
• Typical myotomy length 12-16 cm for Type III disease 6
• Response rate 92% in meta-analyses 6
• Must be performed only at high-volume centers by experienced operators (20-40 procedures needed for competence) 5, 4, 6

If POEM unavailable: Extended laparoscopic Heller myotomy with proximal extension, though results are inferior to POEM for Type III disease 6

Special Considerations for Pediatric Population

Triple A Syndrome (Achalasia-Addisonianism-Alacrima)

• Symptoms develop earlier but diagnosis is frequently delayed 2
• Patients are more underweight at diagnosis and last follow-up 2
• Same treatment principles apply but anticipate more challenging nutritional management 2
• Requires multidisciplinary team including endocrinology for adrenal insufficiency management 7

Post-Treatment Reflux Management

All children require aggressive reflux surveillance and management:

• Mandatory proton pump inhibitor (PPI) therapy immediately post-procedure, particularly after POEM 4, 6, 1
• POEM carries highest reflux risk (10-40% rate of symptomatic GERD or ulcerative esophagitis) and may require indefinite PPI therapy 4, 6
• Post-POEM patients need surveillance endoscopy due to disruption of lower esophageal sphincter 1
• LHM with fundoplication has lower reflux rates but still requires monitoring 5

Critical Management Pitfalls

Avoid Botulinum Toxin as Primary Therapy

• Provides only short-term relief 3
• 85% of children initially treated with botulinum toxin ultimately required myotomy 2
• Should be reserved only when other treatments are contraindicated 8

Anticipate Need for Repeat Interventions

• 29% of children who underwent myotomy required repeat myotomy 2
• 65% of children with initial Heller myotomy required further interventions 2
• Symptoms persist and significantly impact quality of life despite multiple procedures 2

Monitor for Complications

• Suspect perforation if patient develops pain, breathlessness, fever, or tachycardia after any intervention 4
• Watch for pulmonary aspiration, chest infections, persistent dysphagia, and weight loss as these most significantly affect morbidity and mortality 4

Multidisciplinary Team Approach

Optimal management requires coordination between:

• Pediatric gastroenterologist (primary coordinator in 76% of centers) 7
• Pediatric surgeon (involved in 87% of centers) 7
• Radiologist (61% of centers) 7
• Dietician for nutritional support (37% of centers) 7
• Consider psychologist given significant long-term impact on quality of life 7, 2