A 74-year-old woman presented to the emergency department with a three-day history of nausea, vomiting and melena. Her medical history included chronic obstructive pulmonary disease and a curative resection of pancreatic adenocarcinoma with duodenojejunostomy performed three months ago. Her regular medication included torasemide, pantoprazole, pravastatin, duloxetine and olanzapine, while recent intake of other substances was denied. On admission she was hypotensive, hypothermic and she had a heartrate of 93 bpm. The further clinical physical examination was unremarkable. Laboratory testing revealed markedly elevated inflammation parameters while hemoglobin and lactate levels were normal.

Emergency esophagogastroduodenoscopy (EGD) was performed for suspected gastrointestinal bleeding and revealed a circumferential black discoloration of the lower two thirds of the esophagus with a sharp demarcation at the gastroesophageal junction and no involvement of the stomach. Beyond the stomach, the small intestine also exhibited blackened friable mucosa. No active bleeding was detected. (Panel A)

Based on the clinical, laboratory and endoscopic findings, what is the diagnosis? - Acute esophageal necrosis (AEN) accompanied by concurrent small bowel necrosis was established.

AEN, also known as “black esophagus”, is associated with high mortality rates of about 30% [1,2]. AEN is commonly associated with cardiovascular disease, malignancy, hyperglycemia, malnutrition, cocaine use and sepsis.[3,4]. 

Patients commonly present with signs of gastrointestinal bleeding, with the diagnosis typically made via endoscopy. While some cases of AEN have been reported previously, simultaneous small bowel involvement is very rare [2]. The pathogenesis of this phenomenon is incompletely understood and probably results from synergistic effects of ischemic insult and impaired mucosal defense against gastric acid, particularly in the hypoperfused regions supplied by the celiac axis. Further management includes the exclusion of complications such as esophageal perforation, supportive pharmacotherapy with proton pump inhibitors and treatment of comorbidities. 

Subsequent contrast-enhanced tomography showed no signs of perforation or vascular abnormality. The patient was treated with high-dose proton pump inhibitors and broad spectrum antibiotics. A follow-up EGD on day three revealed substantial mucosal healing (Panel B). Enteral nutrition was gradually reintroduced and tolerated well. Inflammatory parameters normalized and the patient was referred to a local hospital for further rehabilitation after 14 days.