Sara Ghoneim, MD1, Shaman Dalal, MD2, Aun Shah, MD1, Marc Landsman, MD1, Annette Kyprianou, MD1
1MetroHealth Medical Center, Case Western Reserve University, Cleveland, OH; 2Case Western Reserve University / MetroHealth Medical Center, Cleveland, OH
Introduction: Acute esophageal necrosis (AEN) or ‘black esophagus’ is a rare disease of the esophagus with an endoscopic prevalence of 0.02%. It generally presents with signs of upper GI bleeding including melena and hematemesis. Its pathophysiology is largely attributed to severe illness states that affect the perfusion of the esophagus. The distal esophagus being least vascular is most commonly affected. Upper endoscopy reveals circumferential black discoloration. Biopsies are taken to confirm necrosis, as well as rule out infection or pigmentation. Management is generally supportive, with endoscopic resolution seen in many cases with correction of the underlying insult.
Case Description/Methods: Three patients with AEN were identified over the last 2 years at our institution. Two females aged 67 and 78 were admitted within the same month in 2019 with epigastric pain and coffee-ground-emesis. Both were found to be in diabetic ketoacidosis (DKA). One of them was diagnosed with CHF with reduced ejection fraction (40%). Both patients had documented T2DM and atherosclerotic disease. Laboratory findings showed elevated serum glucose and anion-gap metabolic acidosis with elevated beta-hydroxyburate. Both underwent EGD which was notable for a circumferential black esophagus that abruptly terminated at the gastro-esophageal junction (fig 1&2). The third patient was a 35-year-old male with poorly-controlled T1DM (A1c 9.1%) who noticed melenic stools 2 days prior and presented with hypovolemic shock. EGD was consistent with black esophagus. Biopsy showed acute inflammatory exudates with necrotic cells and numerous fungal hyphae. He was started on micafungin for two weeks and twice daily PPI. EGD 8 weeks later showed complete resolution of initial findings (fig 3).
Discussion: AEN is a rare cause of upper GI bleed, more commonly reported in men and in patients with underlying vasculopathies who present with severe illness. Peak incidence is in the sixth decade of life. Existing literature reports DKA as a relatively common precipitating factor. All of our patients presented with upper GI bleed secondary to AEN and were in DKA superimposed on low perfusion states due to CHF, atherosclerotic disease and sepsis respectively. Two out of three patients were females. Based on our case series, it appears that DKA, a critical illness in itself, further worsens existing low perfusion states. This can manifest as upper GI bleeding. DKA demands prompt correction, especially in patients with concomitant vasculopathy.
Citation: Sara Ghoneim, MD; Shaman Dalal, MD; Aun Shah, MD; Marc Landsman, MD; Annette Kyprianou, MD. P1238 - BLACK ESOPHAGUS OR ACUTE ESOPHAGEAL NECROSIS: A CASE SERIES AND SINGLE-INSTITUTION EXPERIENCE. Program No. P1238. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.