S4441 Esophageal Intramural Pseudodiverticulosis: A Rare Cause of Esophageal Dysphagia

Introduction: Esophageal intramural pseudodiverticulosis (EIPD) is a rare benign disease of the esophagus in which abnormal dilations of esophageal submucosal mucinous glands appear as multiple outpouchings. It was first described in 1960 and only 200-300 cases have since been reported. We present a case of EIPD in a patient who presented with recurrent food impaction. Case Description/Methods: A 50-year-old man with a history of 6 prior episodes of esophageal food impactions and history of esophageal stricture presented with symptoms of esophageal obstruction. Esophagogastroduodenoscopy demonstrated an impacted food bolus in the upper 3rd of the esophagus which was removed, revealing narrowing of the proximal esophagus with congested mucosa, diffuse exudates and multiple possible sinus tracts. Esophageal dilation was performed and reexamination demonstrated mucosal tears without evidence of perforation. Due to post-procedure chest pain, an esophagram was performed demonstrating EIPD of the thoracic esophagus and esophageal mucosal tears in 3 areas without evidence of full-thickness injury. Patient was discharged on proton pump inhibitor therapy. Biopsies demonstrated severe chronic, mildly active esophagitis in the distal and mid esophagus and severe active esophagitis with presence of fungal elements in the proximal esophagus. Fluconazole was then prescribed. Discussion: The pathophysiology of EIPD is unclear. Two postulated mechanisms are chronic inflammation resulting in obstruction of excretory ducts and chronic irritation of the esophagus leading to fibrosis of the submucosa. EIPD exhibits a bimodal distribution with peaks in the early teenage years and the 6th to 7th decades in adults and is more common in genetic men. Patients typically experience dysphagia due to concurrent development of esophageal strictures. EIPD has been associated with systemic inflammatory diseases, gastroesophageal reflux disease, esophageal candidiasis, alcohol use, tobacco use, diabetes mellitus, HIV, Mallory-Weiss syndrome, Crohn's disease, and corrosive esophageal injury. Abnormalities of esophageal motility including achalasia, hypercontractile esophagus, uncoordinated peristalsis, and hypoperistalsis have also been associated with EIPD. Barium esophagram is the preferred diagnostic imaging for EIPD and is characterized by 1-4 mm flask shaped diverticula. Treatment of EIPD consists of anti-fungal therapy if evidence of candidiasis is appreciated, acid-suppression with proton pump inhibitors, and endoscopic dilation if strictures are present.