Esophageal Intramural Pseudodiverticulosis With Tracking in a Child With Autosomal Dominant Hyper‐IgE Syndrome

Abstract

An 11-year-old boy from Saudi Arabia with autosomal dominant hyper-IgE syndrome presented after an incidental finding of esophageal pneumatosis on chest computed tomography. Barium esophagogram demonstrated intramural tracking of contrast from the mid to distal esophagus suggestive of esophageal intramural pseudodiverticulosis (EIP) with tracking (Fig. 1). Esophagogastroduodenoscopy (EGD) confirmed the presence of a single diverticulum (Fig. 2), and culture of esophageal biopsies grew Saccharomyces cerevisiae and Candida glabrata. Esophageal mucosa showed marked eosinophilia and lymphocytosis, with basal cell hyperplasia. The patient returned home before tailored therapy could be initiated.EIP is a condition in which the esophageal submucosal excretory glands form outpouchings, thought to be secondary to obstruction or dilatation from inflammation or altered motility (1). Findings include flaskshaped outpouchings on barium esophagram, and openings of pseudodiverticula seen on EGD (1,2). Tract formation is rare, and typically indicates more diffuse EIP affecting more of the esophagus (1). Although this patient was without gastrointestinal symptoms, EIP most commonly presents as dysphagia, and carries the risk of perforation and mediastinitis (3,4). In this case, we hypothesize that the presence of infection fostered the formation of EIP. This is the first reported case of EIP in hyper-IgE syndrome and highlights the diagnostic utility of EGD paired with an unusual radiographic finding.