Case Presentation: Acute esophageal necrosis (AEN) is a rare condition which presents as acute-onset gastrointestinal bleeding. AEN is associated with vasculitis, coronary artery disease, malignancy, and other conditions which impair maintenance of the esophageal protective barrier. Complications of AEN include esophageal rupture, perforation, stricture formation, and mediastinitis.
A 64-year-old Caucasian female with an extensive past medical history of hypertension, rheumatoid arthritis, hypothyroidism, chronic kidney disease stage III secondary to renal artery stenosis, collagen vascular disease, methotrexate-induced pancytopenia, and hypothyroidism presented to the emergency department with chief complaint of acute onset coffee-ground emesis over 72 hours. Two months prior to presentation, patient was treated for a Schatzki’s of the distal third of esophagus ring with endoscopic dilatation. Vital signs were stable and physical exam was unremarkable.
Labs at admission indicated hemoglobin and hematocrit values 7.4/23.5, respectively. EGD was obtained the next day, and revealed a black mid and distal esophagus with ulcerated longitudinal necrosis at 20cm extending down to gasto-esohageal junction (GEJ), consistent with acute esophageal necrosis. Biopsies from EGD at 25 and 30 cm revealed absence of viable squamous epithelium, and intensely inflamed submucosa with involvement into muscularis propria, as well as abundant granulation tissue, consistent with a diagnosis of acute esophageal necrosis (AEN).
Patient was managed with IV proton pump inhibitor, was made NPO, and was monitored closely for signs of esophageal rupture. She was advanced from NPO to clear liquid diet. Subsequent imaging revealed no evidence of esophageal rupture or pneumomediastinum.
Discussion: Acute esophageal necrosis, also known as “black esophagus,” is a rare clinical entity characterized by diffuse, circumferential, black- appearing esophageal mucosa, with sparing of the GEJ. Acute esophageal necrosis most commonly involves the distal esophagus. It presents with upper gastrointestinal bleeding, abdominal pain, and hemodynamic instability. The etiology of AEN is poorly understood but is thought to be multifactorial, likely from a combination of transient ischemia, gastric outlet obstruction, vascular pathology, physical deconditioning, and insufficient protective barriers of the esophageal mucosa. Complications of AEN include esophageal perforation with mediastinal infection, esophageal stricture and/or stenosis, and death. Close monitoring for evidence of esophageal rupture is required in such patients as many cases resolve spontaneously with conservative approach to allow esophageal re-epithelization as in our patient.
Conclusions: Clinicians should keep black esophagus on their differential for patients presenting with upper GI bleeding when they have multiple cardiovascular comorbidities, and should consider conservative approach when appropriate.