Esophageal intramural pseudodiverticulosis (EIPD) is a disease of unknown pathogenesis characterized by usually systemic, cystic dilatation of the excretory ducts of esophageal submucosal glands. In this article, I review the epidemiology, clinical manifestations, endoscopic findings, esophagographic findings, and histopathology of EIPD. I also discuss the etiology and possible pathogenesis of EIPD based on my experiences with this disease and a review of the literature. EIPD usually presents with dysphagia in middle-aged individuals. It is often complicated with secondary infections, most commonly candidiasis. On esophagography, EIPD is delineated as small, multiple, flask-shaped outward projections within the esophageal wall. In recent years, EIPD has been mainly diagnosed by endoscopic findings of multiple, localized, small mucosal depressions. The orifices of the "pseudodiverticula" periodically open and close, and excrete mucus onto the mucosal surface. On histopathological examination, the luminal surface of dilated ducts in EIPD is covered by multilayered, hyperplastic epithelial cells, but myoepithelial cells in the glandular acini are well preserved. Treatment of EIPD is usually symptomatic therapy, and prevention of the infectious complications is important. The etiology and pathogenesis of EIPD are largely unknown, but functional abnormalities of autonomic nerve fibers innervating the esophageal glands likely play an important role, since the structures of the glands are basically preserved in this disease.

Esophageal intramural pseudodiverticulosis is an uncommon, idiopathic disorder characterized by multiple small outpouchings protruding from the esophageal lumen. Esophageal intramural pseudodiverticulosis is associated with conditions such as gastroesophageal reflux disease and diabetes mellitus, as well as emergent complications including pneumomediastinum. The most common presenting symptom is dysphagia with associated esophageal stricture formation. While the pathogenesis of EIP has yet to be determined, it is important to bring awareness to this unique disease with distinctive diagnostic findings and treatment options.

In this case, we present a 62-year-old woman who suffered from dysphagia, an inability to tolerate a regular diet, and unintentional weight loss for several years prior to her diagnoses. She was diagnosed by esophagram and esophagogastroduodenoscopy to have esophageal intramural pseudodiverticulosis, complicated by severe stricture formation. Following treatment with sequential dilatation and maintenance H2-blocker therapy, she achieved significant symptomatic improvement.

This case highlights the importance of accurate identification and treatment of an uncommon cause of dysphagia, esophageal intramural pseudodiverticulosis. Treatment includes dilatational therapy, as successfully demonstrated in our patient. Furthermore, treatment is focused on optimizing medical management, as demonstrated in our patient with the addition of an H2-blocker for GERD, or addressing potentially serious underlying causes, such as carcinoma, with surgery.