Acute esophageal necrosis (black esophagus)
- Daniel S Mishkin, MDCM, FASGE, FACG, AGAF
Daniel S Mishkin, MDCM, FASGE, FACG, AGAF
- Boston University, Boston Medical Center
- Granite Medical Group
- Daniel Gelrud, MD
Daniel Gelrud, MD
- Associate Professor of Clinical Medicine
- Florida International University
Acute esophageal necrosis, also known as black esophagus and necrotizing esophagitis, is a rare syndrome characterized by a striking diffuse circumferential black appearance of the esophageal mucosa that almost universally affects the distal esophagus and stops at the gastroesophageal junction [1-5].
This topic will review the epidemiology, clinical manifestations, diagnosis, and management of acute esophageal necrosis. The clinical manifestations, diagnosis, and management of other causes of esophagitis are discussed in detail, separately. (See "Clinical manifestations and diagnosis of gastroesophageal reflux in adults" and "Medical management of gastroesophageal reflux disease in adults" and "Clinical manifestations and diagnosis of eosinophilic esophagitis" and "Treatment of eosinophilic esophagitis" and "Medication-induced esophagitis".)
Acute esophageal necrosis is rare with an estimated prevalence of up to 0.2 percent in autopsy series [6,7]. In endoscopy series, the prevalence of acute esophageal necrosis has ranged from 0.001 to 0.2 percent of cases [7-11]. The incidence of acute esophageal necrosis appears to be more than four times higher in men as compared with women, and patients have a mean age of 68 years at diagnosis [5,8,11].
ETIOLOGY AND PATHOGENESIS
The etiology of acute esophageal necrosis is unclear, but ischemia and gastric outlet obstruction may be inciting events [12-14]. In case reports, acute esophageal necrosis has been associated with broad spectrum antibiotic use, infections (eg, Candida albicans, cytomegalovirus, herpes virus, and Klebsiella pneumoniae), gastric volvulus, a paraesophageal hernia, hyperglycemia, diabetic ketoacidosis, an underlying malignancy, Stevens-Johnson syndrome, prolonged vomiting following alcohol binging, alcoholic hepatitis and lactic acidosis, and aortic dissection [8,11,15-26].
According to the "two hit" hypothesis, there is an initial event (ie, low flow vascular state), which then predisposes the esophageal mucosa to a severe topical injury (ie, by reflux of acid and pepsin). Gastric outlet obstruction leads to an accumulation of fluid in the stomach, which can promote esophageal reflux resulting in direct injury with necrosis. In support of this hypothesis is the observation that temporary reduction of esophageal blood flow can result in extensive esophageal necrosis, which resolves rapidly when flow is restored . Furthermore, acute esophageal necrosis tends to occur in the distal third of the esophagus, which is relatively hypovascular compared with other esophageal segments. Finally, the necrosis of the esophageal mucosa and submucosa, microscopic thrombosis, and rapid recovery are similar to the changes seen in ischemic colitis. (See "Colonic ischemia", section on 'Diagnosis'.)
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