Black esophagus: Acute esophageal necrosis syndrome

Abstract

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.

Black esophagus

“…According to the histological appearance (granulocytes but no other types of leukocytes demarcating the necrotic mucosa, with a lack of hemosiderin deposits) the onset of acute esophageal necrosis had not been survived for more than 3-5 days. The coincidence of black esophagus with duodenal ulcers, as demonstrated here at autopsy, has been described previously [4].…”

“…Perfusion injury and hemodynamic instability due to severe arteriosclerosis, cardiovascular diseases (e.g., cardiac arrhythmias, congestive heart failure), thromboembolic phenomena, acute blood loss, hypothermia and shock may lead to ischemic compromise of the esophagus with acute esophageal necrosis [4]. However, none of the aforementioned conditions were known from the previous history of the deceased or established at autopsy and therefore, the exact pathogenetic mechanisms responsible for the development of the condition remain unclear in the present case.…”

Septic shock-related acute esophageal necrosis and stenosis: three cases of acquired esophageal stenosis presenting a similar clinical course

“…Implicated etiology of AEN is a combination of tissue hypo perfusion mainly caused by shock-related microcirculatory dysfunction and impaired local defense barriers and corrosive injury from gastric contents [2]. The theoretical rationale that the lower part of the esophagus below the level of the bifurcation of the trachea is most susceptible to hypo perfusion is based on the following hypothesis: In contrast to the upper to middle esophagus that have sufficient blood supply from the branches of the bilateral inferior thyroid arteries and the bronchial arteries and vascular communications with the trachea, the blood supply of the lower esophagus is singly supported by few proper esophageal arteries.…”

Acute Esophageal Necrosis Caused by Candidiasis in a Patient with Systemic Lupus Erythematosus

“…The disease has been reported primarily in case reports and is believed to preferentially afflict elderly men (1). The predisposing factors reported for AEN include a history of alcoholism, vascular insult, malnutrition, cardiac risk factors, such as diabetes mellitus, hypertension, and dyslipidemia; multiple risk factors are usually present in affected individuals (1,2). The disease has a characteristic endoscopic appearance wherein the involvement of the distal esophagus is more severe and the discoloration abruptly terminates at the gastroesophageal junction.…”

“…The outcome in these patients depends on the underlying condition. Those who survive the acute episode may develop esophageal stricture (1,2). We report a young woman who had been receiving corticosteroids for lupus nephritis and who presented with hematemesis for which a diagnosis of acute esophageal necrosis was established.…”