Achalasia is a disorder involving the lower esophageal sphincter, in which there is failure of normal relaxation of the sphincter during swallowing. Also, there is absence of the normal proplusive contractions within the body of the esophagus. The result is a functional obstruction of the distal esophagus.

Achalasia is uncommon in childhood, with fewer than 5% of the cases occurring in children. The cause is unknown, and most cases are sporadic. Younger children may present with failure to thrive, recurrent pneumonia, asthma, and chronic cough. Older children may develop weight loss, chest pain, dysphagia, or regurgitation.

Plain radiographs may demonstrate an air-fluid level within the region of the esophagus. Esophagrams demonstrate a dilated esophagus with a tapered distal esophagus, and nonpropulsive contractions are seen.

The differential diagnosis is esophageal stricture, carcinoma and leiomyoma of the esophagus.


Reviewer: Dr. Lynne Ruess, MD, Pediatric Radiology, TAMC