Case Presentation: A 58-year-old man with a history of hypertension, type 2 diabetes with non-adherence to insulin, GERD, and daily alcohol use presented to the emergency department for three days of epigastric abdominal pain and vomiting. He had previously experienced two months of worsening nausea and epigastric fullness with eating accompanied by a 30-pound unintentional weight loss. In the days leading up to admission, the pressure in his epigastrium gradually evolved into a severe burning pain and he began to vomit with oral intake. He was eventually unable to tolerate even small sips of water, prompting him to seek care. On arrival to the ED, the patient was tachycardic to 111 and hypertensive to 188/101. He was ill-appearing with dry mucous membranes. A finger-stick glucose was 244 mg/dl. BMP was notable for bicarbonate 11 mmol/L and anion gap 26 mEq/L. ABG showed ph 7.29, pCO2 24 mmHg, pO2 112 mmHg, bicarbonate 12 mmol/L, and lactate 2.3 mmol/L. The beta hydroxybutyrate was elevated > 4.0 mmol/L. The patient was diagnosed with diabetic ketoacidosis and managed according to institutional protocols with IV fluids and an insulin infusionOn arrival to the floor, the patient’s anion-gap metabolic acidosis was resolving but he continued to report severe epigastric pain. Further evaluation with CT abdomen showed significant mural thickening of the distal esophagus and proximal duodenum along with marked gastric distention (Figure 1). Endoscopy subsequently revealed diffuse black and ulcerated mucosa in the entire esophagus with overlying exudate and multiple ulcers in the proximal duodenum (Figure 2). These findings were consistent with a diagnosis of acute esophageal necrosis (AEN). The patient was kept strictly NPO and treated with IV pantoprazole and empiric piperacillin-tazobactam. Over the next few days, his epigastric pain improved and his diet was gradually advanced. He was eventually discharged home on oral pantoprazole.
Discussion: Although this patient was initially admitted with a diagnosis of DKA, the fact that his initial glucose was < 250 mg/dl, the history of progressively limited oral intake in the weeks leading up to hospitalization, and the failure of abdominal pain and vomiting to improve with resolution of ketosis prompted further investigation. The finding of AEN on endoscopy was unexpected, as this condition is more typically encountered in critically-ill patients with hemodynamic instability. However, AEN has also been reported to occur in non-critical systemic illnesses such as DKA and alcohol intoxication. In this case, the pathophysiology of esophageal necrosis appears to have been uniquely complex. Gastric outlet obstruction from duodenal ulcers likely led to worsening of the patient's chronic esophageal reflux and to poor oral intake. Starvation in conjunction with underlying insulin resistance then resulted in ketoacidosis accompanied by vomiting and profound dehydration. Although frank hypovolemic shock never developed, significant intravascular volume depletion in the context of underlying mucosal compromise and microvascular disease was sufficient to set off a vicious spiral of ischemia, worsening symptoms, and further compromise of esophageal perfusion culminating in necrosis.
Conclusions: This case reinforces the importance of maintaining a broad differential for diabetic patients who present with gastrointestinal symptoms and elevated ketones and highlights AEN as a condition that may be encountered without a background of critical illness.
