Achalasia

Achalasia is a rare neurogenic motility disorder of the esophagus, occurring in approximately 0.11 cases per 100,000 children. The combination of problems (aperistalsis, hypertensive lower esophageal sphincter (LES), and lack of receptive LES relaxation) results in patients having symptoms of progressive dysphagia, weight loss, and regurgitation. Treatment modalities have evolved over the past few decades from balloon dilation and botulinum toxin injection to laparoscopic Heller myotomy and endoscopic myotomy. Most data on achalasia management is extrapolated to children from adult experience. This article describes understanding of the pathogenesis and discusses newer therapeutic techniques as well as controversies in management.