Black Esophagus: Acute Esophageal Necrosis as a Cause of Upper GI Bleed

Abstract

Acute esophageal necrosis (AEN), commonly referred to as “black esophagus”, is a rare clinical entity arising from a combination of ischemic insult seen in hemodynamic compromise, corrosive injury from gastric contents, and decreased function of the mucosal barrier system. This is the case of a 57-year-old female with a history of alcoholic cirrhosis who presented to the emergency department with hematemesis,dizziness and right upper quadrant pain. She was obtunded with icteric sclera, and hypotensive with blood pressure of 87/51 mmHg. Initial laboratory evaluation revealed a HGb of 8.9 gm/dl, platelets 46 k/ul, INR 1.8, a creatinine of 1.5 mg/dl and an alcohol level of 137 mg/dl. Her endoscopy revealed a diffuse circumferential hyperpigmented esophageal mucosa with proximal sparing and a clear demarcation of the GE junction consistent with acute esophageal necrosis. (Image 1) The gastric mucosa was diffusely erythematous consistent with portal gastropathy. No ulcers, varices or bleeding were noted. Biopsy was not taken due to high risk of bleeding and perforation. The patient recovered after treatment with intravenous proton pump inhibitor, intake restriction, and treatment of her hepatic encephalopathy. The reported incidence of AEN is 0.01-0.2%. AEN may arise in the setting of multi-organ dysfunction, hypoperfusion, vasculopathy, sepsis, diabetic ketoacidosis, alcohol intoxication, and malignancy. The hallmark of this syndrome is the development of diffuse circumferential black mucosal discoloration in the distal esophagus. Classic “black esophagus” abruptly stops at the gastroesophageal junction. Biopsy is recommended but not required for diagnosis. Preferential involvement of the distal esophagus could suggest ‘water shed' pattern of ischemia resulting from hypotension coupled with a diminished mucosal defense and impaired esophageal barrier. Common complications include perforation and stricture. Aggressive treatment of underlying medical conditions as well as hemodynamic resuscitation, nil per os, and suppression with high dose IV PPI are imperative. Overall mortality in patients with AEN is 32% and clinicians should maintain a high index of suspicion as early diagnosis and prompt treatment improves outcomes.FigureFigureFigure