Acute esophageal necrosis

Last revised by Bruno Di Muzio on 29 Nov 2022

Acute esophageal necrosis, sometimes known as Black esophagus or esophageal stroke, is a rare entity characterized by patchy or diffuse circumferential black pigmentation of the esophageal mucosa from ischemic necrosis.

It is classically characterized by a striking endoscopic image of diffuse, circumferential, black-appearing, distal esophageal mucosa on oesophago-gastroduodenoscopy (EGD) that stops abruptly at the gastro-esophageal junction.

It reported incidence is difficult to accurately determine but from total reported number of cases in the current literature of <150 1.  There is a greater male predilection 5.

It can have a multifactorial etiology.

The distal third of the esophagus is thought to be most commonly affected (97% of cases) due to poor vascularity. 

It generally carries a poor prognosis 3,5 and present with life-threatening upper gastrointestinal hemorrhage. Most cases are conservatively managed 3. It may lead to esophageal perforation.

It is thought to have been been first reported in 1990 and was then classified as a separate syndrome in 2007.

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