Diffuse Leiomyomatosis of the Esophagus – A Rare Cause of Pediatric Pseudoachalasia.

Abstract

Achalasia is an esophageal motility disorder with progressive dysphagia for liquids and solids. It occurs due to absence or degeneration of myenteric plexus of nerves in the distal esophagus. The term pseudoachalasia refers to achalasia like symptoms and imaging manifestations caused by diseases other than aganglionosis [1]. It can be due to stricture, extrinsic compression, malignant neoplasm or proliferation of smooth muscle cells as in leiomyomatosis. Diffuse leiomyomatosis of the esophagus is a very rare cause of pseudoachalasia and requires total esophagectomy with gastric pull through surgery. We present a rare case of a seven year old boy with diffuse leiomyomatosis of esophagus presenting with pseudoachalasia, aspiration pneumonitis and bronchiectasis secondary to recurrent aspirations.