S2003 Cytomegalovirus (CMV)-Induced Acute Esophageal Necrosis (Black Esophagus) in the Lung Transplant Recipient: A Rarest Combination

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INTRODUCTION: Acute esophageal necrosis (AEN) also known as black esophagus is a rare clinical entity with characteristic endoscopic appearance of diffuse circumferential necrosis of distal esophagus with relative sparing of proximal esophagus. We report a 60-year-old man with a double lung transplant a year ago, who developed cytomegalovirus induced AEN. To the best of our knowledge and literature review, this is the first and only case of CMV related AEN with resistant stricture in double lung transplant recipient. CASE DESCRIPTION/METHODS: A 60-year-old male with advanced COPD status post double lung transplant a year ago presented with acute onset of odynophagia with solids/liquids for two days. CT scan of the chest showed diffuse thickening of distal esophageal wall without evidence of perforation. An upper endoscopy revealed severe diffuse necrotic mucosa extending from 26 cm to 39 cm from the incisors (Figure A). Biopsies showed CMV related acute and chronic inflammation with granulation tissue (Figure B). Patient was kept NPO and managed with intravenous hydration, total parenteral nutrition, ganciclovir along with proton pump inhibitors for aggressive acid suppression. Repeat endoscopic evaluation two weeks later showed complete resolution of necrosis but moderately severe stenosis in the lower third of the esophagus (Figure C) that required serial dilations over the course of 1 year without significant improvement. DISCUSSION: Acute esophageal necrosis is a rare condition with an incidence of 0.01-0.28%. Infection is a major contributor in post-transplant patients with immunosuppressed state. Proposed pathophysiology contributing to AEN includes impaired esophageal mucosal reparative mechanisms seen in chronically debilitated patients along with esophageal ischemia seen in low flow states. Most common presentation is upper gastrointestinal bleeding in up to 90% of cases. EGD is diagnostic with distal esophageal involvement in 97% of cases. Esophageal biopsy aids in exclude/confirm infectious etiology and typically not associated with increased risk of perforation. Treatment is mainly supportive with management of underling etiology. Potential complication includes stricture formation and perforation. AEN should be considered in the differential diagnosis of UGI bleed and dysphagia especially in post-transplant patients. In conclusion, isolated CMV related AEN is extremely rare in lung transplant recipients and this is the first ever case reported in the literature.Figure A.: Initial EGD showing black necrotic esophagus and follow up EGD two weeks later with healing of necrosis along with distal esophageal stricture.Figure B.: Giant cell with inclusion body characteristic of CMV esophagitis.Figure C.: Barium Esophagram showing long tapering distal esophageal stricture.