Acute esophageal necrosis: a rare syndrome

Background. Acute esophageal necrosis, which presents as a black esophagus on endoscopy, is a rare disorder that is poorly described in the medical literature. In this study, we analyze all cases reported to date to define risk factors, clinical presentation, endoscopic features, histologic appearance, treatment, complications, outcome and etiopathogenesis of the disease and to describe a distinct medical syndrome and propose a staging system. Methods. We searched Medline and PubMed from January 1965 to February 2006 for English-language articles using the key words "acute esophageal necrosis," "necrotizing esophagitis," and "black esophagus." Results. A total of 88 patients were reported in the literature during the 40 years, 70 men and 16 women with an average age of 67 years. Patients were generally admitted for gastrointestinal bleeding and cardiovascular event/shock. Patients presented with hematemesis and melena in more than 70% of the cases. Upper endoscopy showed black, diffusely necrotic esophageal mucosa predominantly affecting the distal third of the organ. Necrosis was confirmed histologically in most cases. Complications included strictures or stenoses, mediastinitis/abscesses, and perforations. Overall mortality was 31.8%. Conclusion. This study provides a structured approach to identifying risk factors, diagnosis, and pathogenesis of the acute esophageal necrosis. Risk factors include age, male sex, cardiovascular disease, hemodynamic compromise, gastric outlet obstruction, alcohol ingestion, malnutrition, diabetes, renal insufficiency, hypoxemia, hypercoagulable state, and trauma. Mechanism of damage is usually multifactorial secondary to ischemic compromise, acute gastric outlet obstruction, and malnutrition. Overall, acute esophageal necrosis should be viewed as a poor prognostic factor, associated with high mortality from the underlying clinical disease.