Practical Review: Gurvits Syndrome

Black Esophagus or Acute Esophageal Necrosis

_{Klaus Mönkemüller, MD, PhD, FASGE, FJGES} 

_{Professor of Medicine}

_{Department of Gastroenterology, Carilion Memorial Hospital, Virginia Tech Carilion School of Medicine, Roanoke, USA} 

_{Vivek Kesar, MD}

_{Assistant Professor, Department of Gastroenterology, Carilion Memorial Hospital, Virginia Tech Carilion School of Medicine, Roanoke, USA} 

Figure 1. Acute esophageal necrosis. This patient had ischemia due to massive vasoconstriction due to epinephrine use to resuscitate the patient after a heart attack with cardiogenic shock. Panel C also shows ulcers and necrosis of the duodenum.

Acute esophageal necrosis (AEN), commonly referred to as "black esophagus", is a rare clinical entity also known as Gurvits syndrome (1-3) (Figures 1-3).  Acute esophageal necrosis arises from a combination of ischemic insult seen in hemodynamic compromise and low-flow states, corrosive injury from gastric contents in the setting of dysmotility (esophago-gastroparesis) and gastric outlet obstruction, and decreased function of mucosal barrier systems and reparative mechanisms present in malnourished and debilitated physical states (1). Not uncommonly, diabetic ketoacidosis (DKA) also results in this condition. Less commonly, infections such as aspergillosis may also turn the esophagus black (Table below). 

Table. Etiology and Conditions Associated with Black Esophagus (Esophageal Necrosis)

CategoryFactorsVascularischemia, atherosclerosis, shock, thrombophilia (hypercoagulable condition)Mechanical (severe reflux)nasogastric tube, gastric outlet obstruction, gastric volvulusChemicallye, corrosives, high-percentage ethanol, antibioticsMetabolic/endocrinediabetes mellitus (DM) (diabetic ketoacidosis, gastroparesis), uremiaVasculitis/systemicpolyarteritis nodosa, amyloidosisInfectionsaspergillosis, herpes simplex virus, cytomegalovirusUnderlying co-morbiditiesvasculopathy, atherosclerosis, COPD, DM, cancer, malnutrition, ESRD (end-stage renal disease)

Figure 2. An elderly patient suffering from hemodynamic and septic shock requiring high doses of pressor support developed hematemesis. On EGD ischemic esophagitis was detected.

Figure 2. Additional examples of black esophagus in patients with diabetic ketoacidosis (A, B) and hemodynamic shock (C).

Gurvits syndrome or 'black esophagus' is caused by microvascular thrombosis and neutrophilic inflammation (ischemic necrosis) (2). Microvascular thrombosis is related to various entities, including hemodynamic shock, vasculitis, antiphosolipid syndrome, immunological impairment, malignancy, diabetes mellitus, alcoholism (2) (Table). Histology shows mucosal/submucosal necrosis, dense neutrophilia, edema, sloughed epithelium, and debris.

Therapy of Gurvits syndrome is focused at reversing the underlying clinical conditions, such as restoring hemodynamic perfusion, treating sepsis, and improving oxygenation. The patient is initially kept NPO (nil-per-os), and aggressive intravenous acid suppression with proton pump inhibitors is warranted (1, 2). Complications include perforation with mediastinal infection/abscess, esophageal stricture and stenosis, superinfection, and death, which can be as high as 32% (1, 2). 

References:

1. Gurvits GE, Shapsis A, Lau N, Gualtieri N, Robilotti JG. Acute esophageal necrosis: a rare syndrome. J Gastroenterol. 2007 Jan;42(1):29-38. doi: 10.1007/s00535-006-1974-z. Epub 2007 Feb 16. PMID: 17322991.

2. Gurvits GE. Black esophagus: acute esophageal necrosis syndrome. World J Gastroenterol. 2010 Jul 14;16(26):3219-25. doi: 10.3748/wjg.v16.i26.3219. PMID: 20614476; PMCID: PMC2900712.

3. Diaconu C, Ciocîrlan M, Ilie M, Sandru V, Balaban DV, Plotogea O, Diculescu M. Gurvitis syndrome: the dark shade of hematemesis. Endoscopy. 2020 May;52(5):E181-E182. doi: 10.1055/a-1059-9268. Epub 2019 Dec 2. PMID: 31791097.

No COI by KM or VK with any of the companies/utensils or products mentioned in this article. 

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