Case Presentation: A 55-year-old man with a history of homelessness, poorly controlled type 2 diabetes treated with insulin and complicated by recurrent diabetic ketoacidosis (DKA), peripheral vascular disease and neuropathy with foot ulcers, severe autonomic neuropathy with postural hypotension and recurrent syncope treated chronically with midodrine and fludrocortisone, and chronic heart failure with mid-range ejection fraction, originally presented to the emergency department with chest pain. Ischemic cardiac workup was negative, but a SARS-CoV-2 screen was positive. A CT angiogram of his chest suggested findings of esophagitis but did not show pneumonia or pulmonary embolism. He was treated with carisivimab-indevimab and discharged to a “COVID motel” to isolate. He returned two days later with nausea and vomiting, was diagnosed with gastritis, and discharged with a proton-pump inhibitor.He was admitted two days later with severe encephalopathy and DKA after having stopped injecting insulin due to persistent GI symptoms, including melena. The DKA quickly resolved with fluid resuscitation and subcutaneous insulin, but he had recurrent melena. Endoscopy showed diffuse, black, mucosal discoloration and sloughing of the entire esophagus, consistent with ischemic necrosis.
Discussion: Acute esophageal necrosis (AEN) classically presents as black necrotic mucosa within the distal third of the esophagus, potentially extending proximally and sparing the gastroesophageal junction. This has been described as a rare complication associated with DKA as well as other vascular pathology. Other findings beside gastrointestinal bleeding include leukocytosis and anemia. The pathophysiology likely includes ischemic changes and impaired mucosal defense.Patients with diabetes have many risk factors for esophageal ischemia, including atherosclerotic disease and small vessel disease. Ketoacidosis creates a low-flow vascular state with hypovolemia. Electrolyte derangements and poor nutritional status contribute to poor mucosal defense. Wischnewsky lesions, a gastric finding classically seen on autopsy following hypothermia but also increasingly associated with DKA, may have a similar pathophysiology.Diagnosis is usually confirmed with its characteristic appearance on endoscopy. The friable mucosa increases the risk of esophageal perforation and mediastinitis. During this period, management involves restricting oral intake, avoiding passage of NG tubes, and PPI therapy. Acid reflux and esophageal dysmotility may persist for 5-7 months after AEN resolves. Necrosis appears to resolve on endoscopy after treatment of DKA. Long-term complications include stricture and stenosis. Repeat endoscopy can document resolution of AEN, determine duration of PPI therapy, and allow dilatation of new stricture/stenosis.
Conclusions: Acute esophageal necrosis is a rare, but severe, complication of diabetic ketoacidosis, with a high risk of morbidity and mortality. Signs of upper GI bleeding in a patient being treated for DKA should prompt concern for AEN. Management typically involves proton pump inhibition, avoiding manipulation of the friable necrotic mucosa, and close monitoring for complications like perforation, stricture, and stenosis.
