Abstract

An 11-year-old boy presented to our pediatric pulmonology clinic with a 3-month history of atelectasis evident on

his chest radiography. Breath sounds revealed fine crackles in the right lower zone and rhonchi in the upper zones.

His initial pulmonary function test was compatible with restrictive pulmonary disease. Chest tomography revealed

that the trachea, right intermediate, and middle lobe bronchi were narrowed by megaesophagus. Esophagogram

determined dilatation of the esophagus and “bird-beak” sign in the esophagogastric junction but it was not sufficient

to diagnose. Esophageal manometry which is the gold standard test for achalasia was performed and type 2

achalasia was diagnosed. His symptoms improved following Heller myotomy conducted together with Dor fundoplication.

Although respiratory problems are more common in infants and younger children, atypical respiratory

presentations may also occur during adolescence. Achalasia should be one of the rare differential diagnoses of

pediatric restrictive pulmonary disease.