Figure 1: (A) (Left) Esophagus opened longitudinally to reveal a deeply stained distal esophageal mucosa with an abrupt transition at the gastroesophageal junction. (B) (Above) Histology from blackened esophagus showing full thickness epithelial necrosis, overlying nonspecific black pigment, inflammatory infiltrate, and multiple small caliber thromboemboli (hematoxylin-eosin, original magnification 40x).
What is the cause of death in this case?
with an average age of 67 years. 1,4 The gross pathology of AEN is pathognomonic char- acterized by circumferential black discoloration of the esophageal mucosa, primarily in the distal segment (97%) but occasionally with proximal extension to involve the upper one-third. 1 Notable is the sharp contrast and abrupt transition with normal appearing gastric epithelium at the gastroesophageal junction. 1,4 Gross features are so remark- able that diagnosis is frequently made by esophagogas- troduodenoscopy (EGD) without the requisite need for biopsy. 1,2 Histology, however, is also typical and includes necrosis of the mucosa and submucosa without any remain- ing recognizable viable squamous epithelium. Necrosis into the muscularis layers and full thickness into the adventitia have also been described. Additional findings include a heavy leukocytic infiltrate, deranged muscle fibers, and vascular thrombi. 1,2,4,5 Altenberger et al. also describe a black nonspecific granular pigment that stains positively with periodic acid Schiff (PAS) and negatively for iron. 5 The etiology of AEN is unclear but is thought to be multi-factorial and result from a combination of ischemia, backflow reflux of acidic gastric contents, and impaired local defense barriers. 1,2,4 Several studies have supported a role for ischemia in the pathogenesis of AEN, particularly since the distal esophageal segment is relatively hypovascular compared to the more proximal segments. 1,2,4,5 The histo-
DIAGNOSIS: Acute esophageal necrosis, ischemic and pseudomembranous colitis DISCUSSION Acute esophageal necrosis (AEN) is a rare disorder with an unclear etiology that is also known as “black esophagus.” 1,2 A relatively newly described diagnosis, AEN was first reported by Goldenberg et al. in 1990. 1,3 Affected patients most often present with upper gastrointestinal bleeding, including hematemesis. Other symptoms may in- clude nausea and vomiting, dysphagia, fever, lightheaded- ness, and syncope. AEN occurs more often in patients with multiple co-morbidities, particularly those with diabetes mellitus, malignancies, hypertension, alcohol abuse, coro- nary artery disease, chronic obstructive pulmonary disease (COPD), renal insufficiency, immunosuppression, sepsis, broad spectrum antibiotic use, and aortic dissection. 1,2,4,5 AEN is a rare disorder. Two large autopsy studies have found zero cases in 1,000 consecutive autopsies and a 0.2% frequency in 3,000 total autopsies. 1,6,7 Two retrospective endoscopic studies have estimated the incidence at 0.01% and 0.28%. 1,8-10 Men are affected four times more commonly than women. The disease has been documented in all age groups, but the peak incidence occurs in the sixth decade
J La State Med Soc VOL 166 July/August 2014 189
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