May 2022 – Gastroenterology Case Two

Acute esophageal necrosis.

Acute esophageal necrosis (AEN) is a rare and deadly disease that can cause life-threatening esophageal hemorrhage. AEN is associated with poor nutritional status and multiple comorbidities, including alcoholic cirrhosis, binge drinking, diabetic ketoacidosis, and infection. AEN is characterized by circumferential black discoloration of the distal esophagus with underlying friable hemorrhagic tissue with a sharp transition to normal-appearing mucosa at the gastroesophageal junction. Histologically, severe esophageal mucosal necrosis is seen in AEN. Inflammation and partial destruction of adjacent muscle fibers and blood vessel thrombosis or occlusion may also be seen. The pathophysiology is hypothesized to result from a combination of esophageal mucosal injury and microvascular thrombosis.

Although esophageal variceal rupture can cause acute life-threatening gastrointestinal hemorrhage, especially in alcoholic patients, AEN presents with different gross and histologic findings. Large, dilated submucosal veins that protrude into the esophageal lumen and impart a mottled blue discoloration to the mucosa are seen on gross examination. Histologically, dilated, thin-walled veins in the submucosa are seen with overlying mucosal erosion and are more characteristic of esophageal varices.

Although the ingestion of caustic substances can cause severe esophageal damage with mucosal sloughing and black discoloration, this can be distinguished from AEN based on history as well as the presence of oropharyngeal burns.

Although focal or scattered black discoloration of the distal esophagus can be seen in esophageal melanosis, it is associated with underlying chronic esophagitis. Additionally, melanocytes are seen on histology rather than mucosal necrosis.

References

1. Abdullah HM, Ullah W, Abdallah M, et al. Clinical presentations, management, and outcomes of acute esophageal necrosis: a systemic review. Expert Rev Gastroenterol Hepatol. 2019;13:507.
2. Endo T, Sakamoto J, Sato K, et al. Acute esophageal necrosis caused by alcohol abuse. World J Gastroenterol. 2005;11:5568-5570.
3. Jessurun J, Cui I, Aristi-Urista G: Acute (gangrenous) esophageal necrosis (black esophagus). A rare form of injury with specific histologic features and diverse clinical associations with a common pathogenesis. Hum Pathol, 2019;87:44-50.
4. O'Brien J et al: Management of varices in patients with cirrhosis. Nat Rev Gastroenterol Hepatol. 2013;10(7):402-12.
5. Sharma SS, Venkateswaran S, Chacko A, Mathan M. Melanosis of the esophagus. An endoscopic, histochemical, and ultrastructural study. Gastroenterology. 1991;100:13.