S1909 Black Esophagus: A Syndrome of Acute Esophageal Necrosis

Abstract

INTRODUCTION: Black esophagus, also known as acute esophageal necrosis (AEN) syndrome is a rare entity characterized by patchy or diffuse circumferential black pigmentation of esophageal mucosa from ischemic necrosis and may present with life threatening upper gastrointestinal hemorrhage (UGIH) resulting in high mortality in immunocompromised patients. CASE DESCRIPTION/METHODS: A 56-year-old female with diabetes mellitus (DM), gastroesophageal reflux disease (GERD), and alcohol abuse (8–10 drinks daily) presented with multiple episodes of coffee ground emesis and epigastric abdominal pain for two days. The pain was sharp epigastric, 10/10 in severity, non-radiating, precipitated with ingestion of oral intake, without alleviating factors. On examination, she was tachycardic (120/minute) and hypotensive (80/50 mm of Hg), had oropharyngeal erythema with white spots on its posterior wall, and significant ptyalism. She had epigastric tenderness and unremarkable digital rectal examination. Laboratory workup revealed lactic acidosis (>15 mmol/L), acute kidney injury with creatinine 2.21 mg/dL, leukocytosis (18 k/dL), and transaminitis. A computed tomography (CT) of abdomen and pelvis without contrast was unremarkable. The hospital course was complicated by hematemesis, dysphagia, and melena. After initial resuscitation, esophagogastroduodenoscopy (EGD) was performed which revealed inflammation of the epiglottis, arytenoid cartilages, and dark mucosal pigmentation of distal 2/3 of the esophagus with associated hiatus hernia (Figure 1). A biopsy of esophageal mucosa demonstrated fragments of fibrinopurulent exudate and necrotic tissue with pigment deposition (Figure 2). Evidence of helicobacter pylori, cytomegalovirus, herpes simplex virus, candida albicans infections, and malignancy was not found on further testing. She was managed with strict NPO, total parenteral nutrition, proton pump inhibitors, and analgesics. Serial EGDs showed complete resolution of the lesion in 4 weeks and was transitioned to an oral diet. DISCUSSION: In this case, vascularity of the distal esophagus was compromised due to uncontrolled DM, GERD, and binge alcohol drinking, complicated with acute UGIH due to mucosal tears from persistent vomiting. Acute UGIH is alarming for AEN in patients with multiple co-morbidities and a high index of clinical suspicion is required for pursuing EGD for early diagnosis. Early recognition and aggressive resuscitation are the fundamental principles for the management of AEN and better outcome of the disease.Figure 1.: Distal two-thirds of the esophagus showing black pigmentation of mucosa due to ischemic necrosis.Figure 2.: A biopsy of esophageal mucosa demonstrating fragments of fibrinopurulent exudate and necrotic tissue with pigment deposition (arrow).