Black esophagus: new insights and multicenter international experience in 2014

Abstract

Background: Black esophagus or acute esophageal necrosis (AEN) is a striking medical condition characterized by circumferential black appearing esophageal mucosa affecting various lengths of the organ with nearly universal involvement of the distal esophagus and abrupt transition at the gastroesophageal junction. This syndrome is gaining acceptance as an important cause of upper gastrointestinal hemorrhage in hospitalized patients.

Aims: To better describe clinical features, risk factors, associated conditions, etiology, treatment, complications, and outcome in patients with AEN at various medical centers across the globe.

Methods: We analyze this syndrome as a first international multicenter series of eight patients who presented with AEN. Clinical characteristics were recorded for each case by treating physicians, and data were retrospectively analyzed and compared.

Results: AEN is more common in geriatric males and characteristically (88 %) presents with signs of upper gastrointestinal hemorrhage. Risk factors include alcohol abuse, hypertension, diabetes mellitus, dyslipidemia, chronic kidney disease, malnourishment, and vascular disease. Hypoalbuminemia was universal, while anemia, renal insufficiency, and hyperglycemia were seen in nearly 90 % of the patients. Endoscopically, distal esophageal involvement with various proximal extension and sharp demarcation at the GE junction was seen in all patients. Duodenal pathology was seen in 50 % of the cases. Causes of AEN were largely multifactorial with all patients affected by a combination of ischemic insult, compromised mucosal defenses, and corrosive reflux injury of gastric contents. Treatment with acid suppression and correction of underlying medical conditions was implemented in all patients. Esophageal stricture formation was seen in 25 % of the patients and was associated with concurrent duodenal pathology. Mortality was 12.5 % and unrelated to AEN.

Conclusion: Black esophagus or AEN syndrome is an important cause of upper gastrointestinal hemorrhage with striking endoscopic presentation in a clinically compromised individual with multiple co-morbidities. Structured conservative management with correction of underlying etiology and acid suppression is essential to improved prognosis. Associated duodenal pathology on endoscopy is a reflection of the degree of initial insult and a clinical predictor for potential stricture development. Proximal extension of the black esophagus may be related to the degree of hyperglycemia. Increased awareness of AEN syndrome will lead to early recognition and proper treatment.