title: Enteric neuropathology of congenital intestinal obstruction: A case report.
abstract: 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.
authors.affiliation: Department of Pediatrics, Pediatric Gastroenterology Unit, University of Rome La Sapienza, Rome, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Di Nardo
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Stanghellini
authors.forename: Salvatore
authors.initials: S
authors.lastname: Cucchiara
authors.forename: Giovanni
authors.initials: G
authors.lastname: Barbara
authors.forename: Gianandrea
authors.initials: G
authors.lastname: Pasquinelli
authors.forename: Donatella
authors.initials: D
authors.lastname: Santini
authors.forename: Cristina
authors.initials: C
authors.lastname: Felicani
authors.forename: Gianluca
authors.initials: G
authors.lastname: Grazi
authors.forename: Antonio D
authors.initials: AD
authors.lastname: Pinna
authors.forename: Rosanna
authors.initials: R
authors.lastname: Cogliandro
authors.forename: Cesare
authors.initials: C
authors.lastname: Cremon
authors.forename: Alessandra
authors.initials: A
authors.lastname: Gori
authors.forename: Roberto
authors.initials: R
authors.lastname: Corinaldesi
authors.forename: Kenton M
authors.initials: KM
authors.lastname: Sanders
authors.forename: Roberto
authors.initials: R
authors.lastname: De Giorgio
mesh_terms: D001706:Biopsy; D002675:Child, Preschool; D017615:Enteric Nervous System / Q000473:pathology*; D005260:Female; D006801:Humans; D007415:Intestinal Obstruction / Q000151:congenital* / Q000473:pathology* / Q000601:surgery*; D007418:Intestinal Pseudo-Obstruction / Q000473:pathology* / Q000601:surgery*; D009474:Neurons / Q000648:ultrastructure