Acute esophageal necrosis (AEN) is a rare entity with multifactorial etiology, usually presenting with signs of upper gastrointestinal bleeding.

To systematically review all available data on demographics, clinical features, outcomes and management of this medical condition.

A systematic literature search was performed with respect to the PRISMA statement (end-of-search date: October 24, 2018). Data on the study design, interventions, participants and outcomes were extracted by two independent reviewers.

Seventy-nine studies were included in this review. Overall, 114 patients with AEN were identified, of whom 83 were males and 31 females. Mean patient age was 62.1 ± 16.1. The most common presenting symptoms were melena, hematemesis or other manifestations of gastric bleeding (85%). The lower esophagus was most commonly involved (92.9%). The most widely implemented treatment modality was conservative treatment (75.4%), while surgical or endoscopic intervention was required in 24.6% of the cases. Mean overall follow-up was 66.2 ± 101.8 d. Overall 29.9% of patients died either during the initial hospital stay or during the follow-up period. Gastrointestinal symptoms on presentation [Odds ratio 3.50 (1.09-11.30), P = 0.03] and need for surgical or endoscopic treatment [surgical: Odds ratio 1.25 (1.03-1.51), P = 0.02; endoscopic: Odds ratio 1.4 (1.17-1.66), P < 0.01] were associated with increased odds of complications. A sub-analysis separating early versus late cases (after 2006) revealed a significantly increased frequency of surgical or endoscopic intervention (9.7 % vs 30.1% respectively, P = 0.04)

AEN is a rare condition with controversial pathogenesis and unclear optimal management. Although the frequency of surgical and endoscopic intervention has increased in recent years, outcomes have remained the same. Therefore, further research work is needed to better understand how to best treat this potentially lethal disease.

• Acute esophageal necrosis
• Black esophagus
• Acute necrotizing esophagitis

• Acute esophageal necrosis
• acute necrotizing esophagitis
• black esophagus
• gastrointestinal bleeding

Acute esophageal necrosis (AEN), commonly referred to as “black esophagus”, is a rare clinical entity arising from a combination of ischemic insult seen in hemodynamic compromise and low-flow states, corrosive injury from gastric contents in the setting of esophago-gastroparesis and gastric outlet obstruction, and decreased function of mucosal barrier systems and reparative mechanisms present in malnourished and debilitated physical states. AEN may arise in the setting of multiorgan dysfunction, hypoperfusion, vasculopathy, sepsis, diabetic ketoacidosis, alcohol intoxication, gastric volvulus, traumatic transection of the thoracic aorta, thromboembolic phenomena, and malignancy. Clinical presentation is remarkable for upper gastrointestinal bleeding. Notable symptoms may include epigastric/abdominal pain, vomiting, dysphagia, fever, nausea, and syncope. Associated laboratory findings may reflect anemia and leukocytosis. The hallmark of this syndrome is the development of diffuse circumferential black mucosal discoloration in the distal esophagus that may extend proximally to involve variable length of the organ. Classic “black esophagus” abruptly stops at the gastroesophageal junction. Biopsy is recommended but not required for the diagnosis. Histologically, necrotic debris, absence of viable squamous epithelium, and necrosis of esophageal mucosa, with possible involvement of submucosa and muscularis propria, are present. Classification of the disease spectrum is best described by a staging system. Treatment is directed at correcting coexisting clinical conditions, restoring hemodynamic stability, nil-per-os restriction, supportive red blood cell transfusion, and intravenous acid suppression with proton pump inhibitors. Complications include perforation with mediastinal infection/abscess, esophageal stricture and stenosis, superinfection, and death. A high mortality of 32% seen in the setting of AEN syndrome is usually related to the underlying medical co-morbidities and diseases.

• Acute esophageal necrosis
• Black esophagus
• Acute necrotizing esophagitis
• Ischemia
• Endoscopy
• Gastrointestinal hemorrhage