Acute esophageal necrosis: An uncommon entityAD Sonavane1, D Gupta1, A Ambekar2, A Nagral1
1 Department of Gastroenterology, Apollo Hospitals, Navi Mumbai, Maharashtra, India
2 Department of Pathology, Apollo Hospitals, Navi Mumbai, Maharashtra, India
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpgm.JPGM_635_20
Source of Support: None, Conflict of Interest: None
A 60-years-old man presented to the emergency department with three episodes of hematemesis and an episode of melena over the previous 24 hours. He also complained of giddiness and palpitations. On examination, he appeared pale, diaphoretic and had a heart rate of 115 beats/minute. He was hypotensive and was immediately fluid resuscitated. Systemic examination revealed a non-tender abdomen and a normal respiratory system. His comorbidities included hypertension and type II diabetes mellitus for which he was being treated with telmisartan, atenolol and insulin. Past history was significant for two episodes of stroke 4 years and 1 year back. He was also receiving atorvastatin and aspirin for the same.
Laboratory evaluation revealed haemoglobin of 10.2 g/dl, total leucocyte count of 12,300 cells/cumm and a normal platelet count. His liver and renal functions were within normal parameters. He was kept nil per oral and aspirin was stopped. Pantoprazole infusion, intravenous antiemetics, broad-spectrum antibiotics and oral sucralfate were commenced. After initial hemodynamic stabilization, he underwent an esophagogastroduodenoscopy. The examination revealed an ulcerated and friable esophageal mucosa in upper one-third and circumferentially thickened, necrotic, black-coloured esophageal mucosa in the middle and lower third. The black-appearing esophageal mucosa abruptly ended at the gastro-esophageal mucosa; with a clear demarcation; beyond which normal gastric mucosa was visualized [Figure 1]. The stomach and duodenal mucosa was normal. The stomach contained small quantity of altered blood. Multiple esophageal biopsies were taken. Histopathological evaluation using hematoxylin and eosin stain showed necrotic squamous esophageal mucosa with ulceration and extensive inflammatory cells. Proliferated blood vessels were visualized. Periodic acid-Schiff and Grocott's methenamine-silver stains did not highlight any microorganisms or fungi. There were no atypical cells or granulomas [Figure 2].
Computerized tomogram (CT) of the abdomen revealed circumferential wall thickening in the lower esophagus. There was no evidence of occlusion of left gastric or splenic arteries. Serological evaluation for cytomegalovirus and herpes simplex virus was negative. Blood culture and esophageal biopsy culture did not grow any pathological micro-organisms or fungi after adequate incubation. His general condition gradually improved over the course of his stay in the hospital. There was no further overt gastrointestinal bleeding. He was commenced on a liquid diet that was gradually upgraded to full diet over 3 weeks. He was discharged on sixth day and was doing well at follow-up after 5 months.
Acute esophageal necrosis (AEN) or “black esophagus” or “acute necrotizing esophagitis” is a rare disorder with an incidence ranging between 0-0.2% and predominantly affects males (81%) beyond 65 years of age. First described in 1990 by Goldenberg et al., this medical phenomenon was established as a distinct clinical entity by Gurvits. Its etiopathogenesis is multifactorial and arises from a “two-hit” phenomenon which includes an ischemic insult to the esophagus and impaired mucosal barrier system or a backflow injury from gastric acid and pepsin. Risk factors include cardiovascular disease, hypotension and shock, acute ethanol abuse, gastric outlet obstruction, diabetes mellitus, malignancy, chronic kidney disease, trauma, surgery, burns, lactic and diabetic ketoacidosis, solid organ transplant recipients, hypercoagulable states and chronic pulmonary diseases.
Majority of patients present with upper gastrointestinal bleeding. Presenting symptoms may also include epigastric pain, nausea, dysphagia, abdominal distension, fever and syncope. An esophagogastroduodenoscopy classically shows diffusely black esophageal mucosa, predominantly involving the distal esophagus circumferentially with an abrupt cut-off at the gastro-esophageal junction, beyond which normal gastric mucosa is visualized. The condition can affect any segment of the esophagus or can be panesophageal. Differential diagnoses include malignant melanoma, acanthosis nigricans, esophagitis dissecans superficialis, coal dust deposition, pseudomelanosis, and melanocytosis of the esophagus. Therapy includes treatment of underlying disease, intravenous hydration, packed red blood cell transfusion, nutrition – enteral as tolerated or parenteral alimentation, intravenous proton pump inhibitors and oral sucralfate. Complications include esophageal stricture or stenosis, esophageal perforation, mediastinitis and mediastinal abscess. Overall mortality is very high and associated conditions like malignancy, immunosuppression, cardiovascular disease, chronic pulmonary and kidney disease increases the risk of mortality.
In conclusion, we describe a rare case of an elderly man with multiple comorbidities and catastrophic presentation of AEN who survived with conservative management. Prompt endoscopic diagnosis and efficient management of the underlying co-morbidities can salvage the patient presenting with this condition; which is otherwise associated with high mortality.
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[Figure 1], [Figure 2]