Achalasia

Pediatric achalasia is rare, with an average age at diagnosis of 7 years. Because symptoms overlap with gastroesophageal reflux disease (GERD), diagnosis is often delayed. Patients present with dysphagia with solids and liquids, regurgitation, and a history of failed treatment of GERD. On imaging, the proximal esophagus is dilated with smooth, tapered narrowing distally, forming the classic “bird’s beak” sign. A definitive diagnosis is made with the absence or markedly diminished contractility seen on HRM. Short-term management is performed endoscopically, whereas definitive treatment is surgical.