Journal of the Louisiana State Medical Society
logical appearance of AEN also bears similarities to that which is seen in ischemic colitis, offering added support for the hypothesis that the two diseases may share common mechanistic etiologies. 2 Other diagnoses that need to be entertained upon identifying a black esophagus include infectious agents and toxic ingestions. Infectious esophagitis is more likely when microscopy reveals inclusion bodies, microorganism colonies, and multinucleated giant cells. Caustic ingestions do not display the typical gross pattern of AEN such that there is distal segment involvement with relative sparing of the proximal esophagus and sharp delineation from the gastric mucosa. 1,2,4,5 The mortality rate for AEN is reportedly as high as 32% and is thought primarily to be due to patients’ existing co-morbidities. 1,4 Uncomplicated AEN in a patient without co-morbidities follows a far more indolent clinical course and is potentially reversible with a far lower mortality rate at approximately 6%. 1 The goal for treatment in AENmainly targets treating the patient’s underlying conditions but also includes parenteral nutrition, proton pump inhibitors, H2 blockers, and sucralfate. Healing and resolution of the esophageal mucosa have been observed over a varied time course, ranging from one week to one month following diagnosis. Perforation of the esophagus is the most serious primary complication in AEN, reported to occur in 7% of cases; but stricture and stenosis are also described in another 10% of cases. 1,4 In conclusion, acute esophageal necrosis or AEN is a rare condition with a high mortality rate that occurs most often in patients with multiple co-morbidities. The charac- teristic black stained esophageal mucosa, circumferentially favoring the distal segment, is easily seen on endoscopic visualization. The discoloration abruptly terminates at the gastroesophageal junction but may extend proximally into the mid and even proximal one-third. Histology of the involved segment shows no viable squamous mucosa, prominent necrosis, or overlying black granular pigment and small caliber thromboemboli. The etiology is unclear but is hypothesized to be a combination of ischemia, reflux of gastric acids, and impaired barrier defenses. The high mortality rate and poor prognosis are due in large part to the patient’s co-morbidities, and treatment is aimed at treating the coexistent conditions and maintaining hemodynamic stability in the patient. The case illustrated here demonstrates the classic clini- cal presentation and typical gross and microscopic pathol- ogy of AEN. Even though retrospective historical review offers the possibility that the decedent’s complaints upon his prior admission may have suggested AEN, a remark- ably high index of suspicion, and EGD would have been required in order to have diagnosed this exceedingly rare and underappreciated pathologic entity. Still further, this patient’s multiple significant comorbidities, which included two primary incidental carcinomas, chronic lung disease and prior alcohol abuse as well as the more recent evolution of an ischemic bowel and overlying pseudomembranous colitis
clearly had a substantial contribution to his fatal outcome.
ACKNOWLEDGEMENTS The authors would like to gratefully acknowledge the support of the Orleans Parish Coroner’s Office: Dr. Jeffrey Rouse and Chief Investigator John Gagliano for providing the case material for this report. REFERENCES 1. Gurvits GE. Black esophagus: Acute esophageal necrosis syndrome. World J Gastroenterol . 2010 Jul 14; 16(26):3219–25. 2. Mishkin, D, Gelrud D. “Acute Esophageal Necrosis.” UpToDate. Ed. Shilpa Grover. N.p., 20 Jan. 2014. Web. 01 July 2014. 3. Goldenberg SP, Wain SL, Marignani P. Acute necrotizing esophagitis. Gastroenterology . 1990 Feb;98(2):493–6. 4. Gurvits GE, Shapsis A, Lau N, et al. Acute esophageal necrosis: a rare syndrome. J Gastroenterol . 2007 Jan; 42(1):29–38. 5. Altenburger DL, Wagner AS, Li S, et al. A case of black esophagus with histopathologic description and characterization. Arch Pathol Lab Med . 2011;135(6):797–798. 6. Postlethwait RW, Musser AW. Changes in the esophagus in 1,000 autopsy specimens. J Thorac Cardiovasc Surg . 1974; 68:953–956. 7. Etienne JP, Roge J, Delavierre P, et al. Esophageal necrosis of vascular origin. Sem Hop . 1969;45:1599–1606. 8. Lacy BE, Toor A, Bensen SP, et al. Acute esophageal necrosis: report of two cases and a review of the literature. Gastrointest Endosc . 1999;49:527–532. 9. Moretó M, Ojembarrena E, Zaballa M, et al. Idiopathic acute esophageal necrosis: not necessarily a terminal event. Endoscopy. 1993;25:534–538. 10. Augusto F, Fernandes V, Cremers MI, et al. Acute necrotizing esophagitis: a large retrospective case series. Endoscopy . 2004;36:411–415.
From the Department of Pathology at Louisiana State University School of Medicine in New Orleans. Dr. Tsao is a third-year Pathology Resident; Ms. Thomas is a fourth-year Medical Student at the University of Queensland in Brisbane, Australia; and Dr. McGoey is an Associate Professor of Pathology and Residency Program Director.
190 J La State Med Soc VOL 166 July/August 2014
Made with FlippingBook - Online catalogs