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

Research output: Contribution to journalArticlepeer-review


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 pseudoobstruction.

Original languageEnglish
Pages (from-to)5229-5233
Number of pages5
JournalWorld Journal of Gastroenterology
Issue number32
Publication statusPublished - Aug 28 2006


  • Chronic intestinal
  • Congenital intestinal obstruction
  • Enteric nervous system
  • Enteric neuropathy
  • Ladd's band
  • Pseudo-obstruction

ASJC Scopus subject areas

  • Gastroenterology


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