1743 From Red to Black: A Deadly Case of Acute Esophageal Necrosis

Abstract

INTRODUCTION: Black Esophagus, or acute esophageal necrosis (AEN), is a rare but deadly clinical syndrome appearing as diffuse circumferential black necrotic mucosa on esophagogastroduodenoscopy (EGD). It is associated with comorbidities that cause hypoperfusion and ischemia to the esophageal mucosa. It has a reported incidence rate of about 0.001% to 0.2%. We report a case of black esophagus in a patient with fulminant hepatic failure related to nonalcoholic steatohepatitis (NASH) cirrhosis. CASE DESCRIPTION/METHODS: A 75-year-old female with a history of diabetes mellitus, hypertension, gastroesophageal reflux disease, NASH-related cirrhosis, and GI bleeding due to esophageal varices presented to the hospital with altered mental status and increased lethargy of 2 weeks’ duration. Her initial lab workup revealed a calculated MELD score of 36. She was diagnosed with an NSTEMI and hepatic encephalopathy in the setting of fulminant hepatic failure. During her hospital stay, she developed coffee ground emesis and melena with resultant drop in hemoglobin requiring transfusion with multiple units of blood. An EGD performed in order to find the source of her apparent upper GI bleeding revealed diffuse circumferential black discoloration of the mucosa at the middle and inferior third of the esophagus demarcated clearly from the normal-appearing mucosa of the cardia at the gastroesophageal junction (Figure 1). Unfortunately, she developed hepatorenal syndrome due to worsening liver function. Due to the poor prognosis, the patient’s family chose to request “do not resuscitate” (DNR) status. Severe hypoxia failing noninvasive positive-pressure ventilation ultimately led to cardiac arrest and death. DISCUSSION: This case emphasizes the need to include black esophagus on the differential of upper GI bleeding in a patient with a history of esophageal varices and NASH cirrhosis and highlights the high mortality rate, estimated at about 32%, associated with this condition. Documented cases exist of black esophagus in patients with alcohol-related cirrhosis, but we found no others reporting this condition in a patient with NASH-related cirrhosis. There is no direct treatment for black esophagus; recommendations are to treat comorbidities, suppress acid production, and optimize vascular perfusion. Complications include stricture, infection, perforation and death. This case clearly demonstrates that early detection through endoscopy and active management of its underlying cause is critical to improving patient outcomes.