Case Report
Copyright ©2006 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Dec 28, 2006; 12(48): 7874-7877
Published online Dec 28, 2006. doi: 10.3748/wjg.v12.i48.7874
Sporadic ganglioneuromatosis of esophagogastric junction in a patient with gastro-esophageal reflux disorder and intestinal metaplasia
Richard Siderits, Iman Hanna, Zahid Baig, Janusz J Godyn
Richard Siderits, Iman Hanna, Janusz J Godyn, Department of Pathology, RWJ University Hospital-Hamilton, United States
Zahid Baig, Hamilton Gastroenterology Group, RWJ University Hospital-Hamilton, United States
Correspondence to: Richard Siderits, MD, FCAP, FAPWCA, MIAC, Assistant Professor of Pathology and Laboratory Medicine, UMDNJ-Robert Wood Johnson Medical School, Robert Wood Johnson University Hospital-Hamilton, One Hamilton Health Place 08690, United States. rsiderits@verizon.net
Telephone: +1-609-5846741 Fax: +1-609-5846439
Received: April 14, 2006
Revised: April 28, 2006
Accepted: November 23, 2006
Published online: December 28, 2006
Abstract

A 58-year-old female with a recurrent history of upper abdominal pain and intermittent dysphagia underwent endoscopic evaluation that demonstrated an irregular and nodular esophago-gastric (EG) junction and gradeIerosive esophagitis. Biopsies showed prominent intestinal metaplasia of Barrett’s type without dysplasia, chronic inflammation and multiple aggregates of large cells within the mucosal lamina propria, some with spindle shaped nuclei. Immunohistochemistry stains for keratins AE-1/AE-3 were negative, while S-100 and NSE were positive. This, together with routine stains, was diagnostic for mucosal ganglioneuromatosis. The background of chronic inflammation with intestinal type metaplasia was consistent with long-term reflux esophagitis. No evidence of achalasia was seen. Biopsies of gastric antrum and fundus were unremarkable, without ganglioneural proliferation. Colonoscopy was unremarkable. No genetic syndromes were identified in the patient including familial adenomatous polyposis and multiple endocrine neoplasia type IIb (MEN IIb). Iansoprazole (Prevacid) was started by oral administration each day with partial relief of symptoms. Subsequent esophagogastroscopy repeated at 4 mo showed normal appearing EG junction. Esophageal manometry revealed a mild non-specific lower esophageal motility disorder. Mild motor dysfunction is seen with gastro-esophageal reflux disease (GERD) and we feel that the demonstration of localized ganglioneuromatosis was not likely related etiologically. In the absence of findings that might suggest neural hypertrophy, such as achalasia, the nodular mucosal irregularity seen with this instance of ganglioneuromatosis may, however, have exacerbated the patient’s reflux.

Keywords: Manometry; Motility; Lower esophago-gastric junction; Esophagus; Ganglioneuromatosis