A Case of Acute Esophageal Necrosis (AEN) in a Hypothermic Patient: The Grave Prognosis of the Black Esophagus

Abstract

Introduction: Acute Esophageal Necrosis (AEN) is a syndrome characterized by diffuse, necrotic, circumferentially black distal esophageal mucosa on esophagogastroduodenoscopy (EGD) that ends at the gastroesophageal junction (GEJ).The exact etiology is unknown, with hypoperfusion predisposing the less vascularized distal esophagus to acute necrosis plays a rule. 70% of AEN cases present with upper gastrointestinal bleeding (UGIB) and melena. We describe a case of AEN caused by severe hypothermia in an alcoholic patient presenting with UGIB. Case Report: 62 year-old lady with history of liver cirrhosis secondary to heavy alcohol intake presented with change in mental status and hematemesis. Vital signs were consistent with temporal temperature of 29.9 C, heart rate of 61 bpm, and blood pressure of 86/49. Bedside pulse oximetry recorded 84% on room air. She was confused, icteric with distended abdomen, and bilateral lower extremity pitting edema. She was admitted to the intensive care unit after intubation. Hemogram showed a hemoglobin of 5.1 g/dL, platelet count of 62, INR 7.2, total bilirubin 8.9, AST/ ALT 154/59, albumin 2.1. Model for End stage Liver Disease (MELD) score at presentation was 33. Patient was resuscitated with warm crystalloid fluids and 2 units of packed RBCs, and started on Octreotide and Pantoprazole drip. EGD showed normal looking proximal third of the esophagus, with diffuse mucosal ulceration of the middle and lower third of the esophagus (figures 1, 2) without varices, and sharp demarcation of affected areas noted in the GEJ (figure 3). Patient's clinical condition deteriorated over the next 24 hours and she deceased despite aggressive supportive therapy with vasopressors, packed RBC, platelets, and fresh frozen plasma transfusion.Figure 1Figure 2Figure 3Discussion: AEN is a rare syndrome with significant morbidity and mortality. It is found in patients undergoing upper endoscopy for evaluation of UGIB. EGD shows diffuse, circumferential, black pigmentation of the distal esophagus, ending sharply at the Z-line of the GEJ. Medical management is supportive with aggressive hemodynamic support, intravenous proton pump inhibitors, and treatment of underlying illness. Mortality rates in patients with AEN reported to range from 13 to 35%. Over 90% of death cases were secondary to their underlying serious illness. In our case, the patient's clinical condition continued to deteriorate due to multi-organ failure despite aggressive medical intervention.