INTRODUCTION: AEN is a rare disease with an incidence ranging from 0.01% to 0.28% of all upper GI endoscopies. AEN occurs in the setting of underlying critical illnesses. Upon review of the English literature, only 14 cases of AEN associated with DKA have been published over the past 50 years. CASE DESCRIPTION/METHODS: A 61 yo M with a PMH: HTN, HTN, GERD, ETOH abuse, and obesity presented to the hospital with severe SOB, worsening mid-back pain, and a 1.5 wk hx of GERD symptoms. T 97.8 F, BP 183/85, HR 141, RR 31/min. BG of 833 mg/dL, arterial blood gas (ABG) pH of 7.164, pCO2 of 11.1 mmHg, HCO3 of 4 mEq/L, and PO2 of 128 mmHg with an anion gap of 25, serum Beta-Hydroxybutyrate of 4.9 mmol/L, 2+ urine ketones. (WBC) of 30.2 K/uL, Hb 13.5 K+ 5.8, Cr: 2.7. Admitted for DKA on day 2 of his hospital stay he developed N/V and hematemesis. He denied melena. On day 3 he had hematemesis and complained of feeling that food was getting stuck in his mid-chest upon swallowing. On day 4 his Hb dropped from 12.8 to 10.7 g/dL and he began to complain of chest pain and discomfort. (EGD) showed severe necrotizing esophagitis along entire length of esophagus with normal stomach and duodenum. Patient was started on oral (PPI) therapy and oral sucralfate therapy. DKA resolved, anion gap closed, AKI resolved, WBC normalized, and he was weaned off the insulin infusion. He was discharged on hospital day 8 with 6 weeks of oral PPI and sucralfate therapy. DISCUSSION: The pathogenesis is hypothesized to be due to the distal portion of the esophagus being a watershed area due to lower levels of vascularization compared to the upper and middle portions. In one case series, 4 out of 16 cases (25%) of AEN occurred in the setting of diabetic ketoacidosis (DKA), and 14% of cases of DKA involved AEN. AEN is more likely to occur in men than women with a 4:1 ratio, and the ages of presentation ranged from 19-91 with a mean of 68 years. It is diagnosed by upper endoscopy which shows circumferential black necrosis in the distal third of the esophagus that may extend along the length of the esophagus into the upper third but will not extend into the gastric mucosa. Mortality is as high as 32% and the mortality specific to AEN is closer to 6%. Complications can include esophageal perforation, mediastinitis, and abscess formation in up to 6%. Late complications include esophageal strictures and stenoses which are seen in 10.2% of cases and appear as early as 7-12 days. AEN is typically managed with high dose PPI and sucralfate.
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