Introduction

Esophageal achalasia is an uncommon pediatric surgical condition characterized by abnormality of, specifically failure of, the distal esophagus to relax with swallowing, associated with abnormal esophageal motility. The initial case description of achalasia was reported in 1674 by Sir Thomas Willis who treated the patient with esophageal bougienage using a whalebone dilator. The incidence of achalasia in children under 15 years of age has been reported at between 0.02 and 0.31 per 100 000 in Ireland and Britain. Approximately one-fifth of children with achalasia will present during infancy with symptoms of regurgitation, cough, aspiration, or failure to thrive; however, definitive diagnosis and subsequent treatment is rarely accomplished in infancy as these symptoms may be confused with feeding difficulty or gastroesophageal reflux. Older children and adolescents with achalasia typically present with dysphagia, substernal chest pain, regurgitation and weight loss.

Treatment of esophageal achalasia is aimed at resolving the clinical symptoms of dysphagia, odynophagia, and regurgitation. This is accomplished by reducing the resting lower esophageal sphincter (LES) pressure by either medical or surgical means. There is no currently available treatment that directly reverses the pathophysiologic mechanism responsible for achalasia in children. As such, all treatment modalities are aimed at symptom control by reducing the effective LES resting pressure. Rendering the LES incompetent allows the esophagus to empty more readily via gravity and residual esophageal motility. Improved esophageal emptying should alleviate or greatly reduce symptoms related to distal esophageal obstruction.