Case Report
Copyright ©2006 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Aug 28, 2006; 12(32): 5229-5233
Published online Aug 28, 2006. doi: 10.3748/wjg.v12.i32.5229
Enteric neuropathology of congenital intestinal obstruction: A case report
Giovanni Di Nardo, Vincenzo Stanghellini, Salvatore Cucchiara, Giovanni Barbara, Gianandrea Pasquinelli, Donatella Santini, Cristina Felicani, Gianluca Grazi, Antonio D Pinna, Rosanna Cogliandro, Cesare Cremon, Alessandra Gori, Roberto Corinaldesi, Kenton M Sanders, Roberto De Giorgio
Giovanni Di Nardo, Salvatore Cucchiara, Department of Pediatrics, Pediatric Gastroenterology Unit, University of Rome “La Sapienza”, Rome, Italy
Giovanni Di Nardo, Vincenzo Stanghellini, Cristina Felicani, Rosanna Cogliandro, Cesare Cremon, Alessandra Gori, Roberto Corinaldesi, Roberto De Giorgio, Department of Internal Medicine and Gastroenterology and Centro di Ricerca Biomedica Applicata (CRBA), Rome, Italy
Gianandrea Pasquinelli, Donatella Santini, Department of Pathology and Department of Surgery and Transplantation, University of Bologna, Bologna, Italy
Gianluca Grazi, Antonio D Pinna, Kenton M Sanders, Department of Physiology and Cell Biology, University of Nevada School of Medicine, Reno, Nevada, United States
Author contributions: All authors contributed equally to the work.
Supported by the Italian Ministry of Education, University and Research (CCOFIN Project No. 2004062155 to RDeG, 2004055120 to GB and 2003064378 to RDeG, GB and VS)
Correspondence to: Roberto De Giorgio, MD, PhD, Department of Internal Medicine and Gastroenterology, St.Orsola-Malpighi Hospital,Via Massarenti 9 - 40138 Bologna, Italy.
Telephone: +39-51-6363558 Fax: +39-51-345864
Received: April 7, 2006
Revised: May 15, 2006
Accepted: May 22, 2006
Published online: August 28, 2006

Experimental evidence indicates that chronic mechanical sub-occlusion of the intestine may damage the enteric nervous system (ENS), although data in humans are lacking. We here describe the first case of enteric degenerative neuropathy related to a congenital obstruction of the gut. A 3-year and 9-mo old girl began to complain of vomiting, abdominal distension, constipation with air-fluid levels at plane abdominal radiology.

Her subsequent medical history was characterized by 3 operations: the first showed dilated duodeno-jejunal loops in the absence of occlusive lesions; the second (2 years later) was performed to obtain full-thickness biopsies of the dilated intestinal loops and revealed hyperganglionosis at histopathology; the third (9 years after the hyperganglionosis was identified) disclosed a Ladd’s band which was removed and the associated gut malrotation was corrected. Repeated intraoperative full-thickness biopsies showed enteric degenerative neuropathy along with reduced interstitial cells of Cajal network in dilated loops above the obstruction and a normal neuromuscular layer below the Ladd’s band.

One year after the latest surgery the patient tolerated oral feeding and did well, suggesting that congenital (partial) mechanical obstruction of the small bowel in humans can evoke progressive adaptive changes of the ENS which are similar to those found in animal models of intestinal mechanical occlusion. Such ENS changes mimic neuronal abnormalities observed in intestinal pseudo-obstruction.

Keywords: Enteric neuropathy, Chronic intestinal pseudo-obstruction, Congenital intestinal obstruction, Ladd’s band, Enteric nervous system