Acute Esophageal Necrosis or Black Esophagus in the Setting of Diabetic Ketoacidosis

Abstract

Acute esophageal necrosis (AEN) or black esophagus is a rare cause of mortality in patients with gastrointestinal bleeding. We present a case of a 54-year-old female who presented with diabetic ketoacidosis (DKA) and developed melena eventually attributed to AEN. The esophagogastroduodenoscopy (EGD) identified severe inflammation with black discoloration consistent with acute esophageal necrosis in the middle and lower esophagus. The patient was managed with intravenous pantoprazole and total parenteral nutrition (TPN) until she was able to tolerate an adequate diet. Black esophagus should be added to the differential diagnosis of patients with DKA who develop gastrointestinal bleeding. This need is stressed by the fact that early treatment is essential to reducing complications and mortality associated with the condition.

Introduction

Acute esophageal necrosis or black esophagus is a rare cause of gastrointestinal bleeding associated with significant mortality. Classic presentation includes circumferential black necrosis of the esophagus, most often distally, that abruptly stops at the gastroesophageal junction on esophagogastroduodenoscopy (EGD). Biopsy of the esophagus can further show necrosis of the mucosa, with possible extension into the submucosa, but is not mandatory for diagnosis. ¹ Incidence ranges between 0.05% and 0.28%.^2-4 Common risk factors include male gender, cardiovascular disease, renal insufficiency, diabetes, gastric outlet obstruction, sepsis, and malnutrition.^1,5,6 We report a case of a 54-year-old female with distal acute esophageal necrosis (AEN) that presented initially with diabetic ketoacidosis (DKA) and developed melena during hospitalization.

Case Description

A 54-year-old female with uncontrolled Type 1 diabetes mellitus complicated by end-stage renal disease, neuropathy, retinopathy, and gastroparesis, hypertension, and hyperlipidemia presented to the emergency department with an altered mental status after being found down in her home. The patient reported persistent nausea and vomiting for several weeks and was unable to give herself insulin the previous night. The patient had no prior gastrointestinal bleeds, dysphagia, or odynophagia. History was significant for an EGD 2 weeks prior to presentation that only demonstrated gastritis. She was hemodynamically stable on presentation, with an unremarkable physical exam including normal bowel sounds and a nondistended, nontender abdomen. Laboratory testing revealed elevated serum glucose of 1149 mg/dl, an anion gap of 34 mmol/L, potassium of 8 mmol/L, bicarbonate of 7 mmol/L, and white blood count of 24 × 10³/cmm. The patient was diagnosed DKA and admitted to the intensive care unit for further management. She was started on insulin drip and the next day DKA resolved.

After being downgraded from the ICU, she began reporting dysphagia and dyspepsia, eventually developing melena the same day. Hemoglobin dropped from 11.0 to 10.2. Empiric vancomycin and piperacillin/tazobactam were also started. The patient was placed on nothing by mouth (NPO) precautions and started on intravenous pantoprazole 40 mg twice a day. An esophagogastroduodenoscopy (EGD) the following morning revealed severe inflammation with black discoloration consistent with acute esophageal necrosis in the middle and lower esophagus, and erythematous duodenitis (Figure 1). No biopsies were taken during the procedure due to possible false lumen. Afterward, she was started on sucralfate and a clear liquid diet, in addition to intravenous pantoprazole 40 mg twice daily.

Figure 1.

Esophagogastroduodenoscopy (EGD) images showing severe, ulcerative, necrotic, circumferential esophagitis in the middle and lower third esophagus above the gastroesophageal junction (A-D). Normal Esophagus at the gastroesophageal junction (E, F).

Chest x-ray ruled out mediastinitis or perforation. Empiric vancomycin and piperacillin/tazobactam that were initiated initially were discontinued when all cultures were negative and mediastinitis and perforation were ruled out.

Over the next several days, the patient gradually advanced her diet, but experienced persistent melena and odynophagia. After days of poor appetite, total parenteral nutrition (TPN) with NPO precautions were initiated as the risks of infection and perforation were too great with a nasogastric tube. After 6 days and tapering of pain medication, the patient began to tolerate food and TPN was discontinued. The patient was discharged from the hospital with a repeat EGD scheduled in 3 months.

Discussion

This case details black esophagus in a patient with many of the risk factors including diabetes, renal insufficiency, gastroparesis, and potential sepsis. While the pathophysiology is likely multifactorial, it is hypothesized that damage to the mucosal tissue from gastric reflux and tissue hypoperfusion are involved.^7,8 The distal esophagus is especially at risk given the proximity to the gastroesophageal junction and watershed blood flow. While this patient was not diagnosed with gastroesophageal reflux, she did have a diagnosis of gastroparesis secondary to type 1 diabetes mellitus. Gastric stasis can mimic the pathology of reflux and predispose the patient to develop AEN. DKA can also predispose the patient to developing hypoperfusion of the esophagus from osmotic dehydration. The patient’s history of ESRD is another risk factor for developing AEN as it could further contribute to vascular disease, including that of the esophagus.

Common complaints of AEN on presentation include epigastric pain, nausea, hematemesis, or melena. ¹ The patient in this case primarily experienced melena and nausea. Esophagogastroduodenoscopy is important in diagnosis of AEN through visualization of the necrotic esophagus. Biopsy can also aid in confirming the diagnosis but is not essential, especially if the patient is at higher risk of perforation or infection as in this case. When biopsy is available, the tissue sample can demonstrate various findings including black discoloration and necrosis of the esophagus that may or may not extend beyond the mucosa.^1,9 Biopsy can assist in ruling out potential complication by superimposed infection. Other complications of AEN can include perforation and esophageal stricture. ¹⁰ Such findings can require immediate stabilization and repair, or potential delayed endoscopic dilation treatment, respectively.

Main goals of treatment is to control the underlying pathophysiology causing necrosis. In this patient, intravenous proton pump inhibitors help control the acidic reflux component by decreasing acid production. Intravenous fluids and treatment of the DKA help control the ischemic component. While blood loss was not a major concern in this case, in patients with concerning blood loss, transfusions are another method of addressing hypoperfusion. Antimicrobial treatment is also indicated when infection is identified and should be tailored to the specific microbe isolated. ¹

The prognosis AEN in the context of DKA can vary considerably based on several key factors. Acute esophageal necrosis is a rare condition, and its occurrence within the setting of DKA adds complexity to the prognostic outlook. The severity and extent of esophageal necrosis play a critical role, with severe cases associated with a higher risk of complications and poorer prognoses. The most serious acute complication is esophageal perforation, which leads to high morbidity and mortality rates.^10,11 Stricture formation, which complicates around 10% of cases, is the most common long-term sequalae of AEN. ¹ Moreover, AEN can give rise to other severe complications, such as mediastinitis and sepsis, which can substantially worsen prognosis and necessitate further interventions. The long-term prognostic implications of AEN in the context of DKA remain insufficiently documented, underlining the need for continued patient monitoring and research in this clinical scenario. Collaborative, multidisciplinary care involving specialists from gastroenterology, endoscopy, intensive care, and diabetology is instrumental in addressing both acute conditions and underlying diabetes management to optimize patient outcomes.^10,12

Conclusion

Odynophagia and gastrointestinal bleeding in the setting of DKA and ESRD should raise concern for black esophagus. Early diagnosis via an EGD with or without biopsy, and treatment should be prioritized to reduce mortality in patients with hemodynamic instability and acute gastrointestinal bleeds. Additionally, empiric antibiotics should be considered if there is concern for perforation and/or infection. Early diagnosis and treatment of underlying etiology are the vital steps in management to avoid long-term complications.