PURPOSE: We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells. METHODS: Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded. RESULTS: We identified 73 patients born at term. Twenty-seven did not need a rectal biopsy. Twenty-one had biopsy proven Hirschsprung's disease, while 17 had a normal rectal biopsy. Eight patients, all of whom presented with severe abdominal distension, showed immature ganglion cells. Seven had failed to pass meconium after birth. X-rays in all patients showed distended loops of bowel. Two neonates underwent an emergency laparotomy and a stoma. A repeat biopsy at 3 months showed maturation of ganglion cells and the stoma was reversed. Rectal biopsy was repeated in two other patients 2-9 months after the first biopsy and showed mature ganglion cells. At follow-up, one patient still suffers from severe constipation. Seven are asymptomatic now, including the two patients who needed a stoma. CONCLUSION: Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.
PURPOSE: We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells. METHODS: Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded. RESULTS: We identified 73 patients born at term. Twenty-seven did not need a rectal biopsy. Twenty-one had biopsy proven Hirschsprung's disease, while 17 had a normal rectal biopsy. Eight patients, all of whom presented with severe abdominal distension, showed immature ganglion cells. Seven had failed to pass meconium after birth. X-rays in all patients showed distended loops of bowel. Two neonates underwent an emergency laparotomy and a stoma. A repeat biopsy at 3 months showed maturation of ganglion cells and the stoma was reversed. Rectal biopsy was repeated in two other patients 2-9 months after the first biopsy and showed mature ganglion cells. At follow-up, one patient still suffers from severe constipation. Seven are asymptomatic now, including the two patients who needed a stoma. CONCLUSION: Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.
Authors: S E Kenny; J M Vanderwinden; R J Rintala; M G Connell; D A Lloyd; J J Vanderhaegen; M H De Laet Journal: J Pediatr Surg Date: 1998-01 Impact factor: 2.545
Authors: A Kubota; K Imura; M Yagi; H Kawahara; S Mushiake; M Nakayama; S Kamata; A Okada Journal: Eur J Pediatr Surg Date: 1999-12 Impact factor: 2.191
Authors: Y Tatekawa; H Kanehiro; H Kanokogi; Y Nakajima; E Nishijima; T Muraji; Y Imai; C Tsugawa; A Toyosaka; H Nakano Journal: Pediatr Surg Int Date: 2000 Impact factor: 1.827