Neonatal functional intestinal obstruction and the presence of severely immature ganglion cells on rectal biopsy: 6 year experience.
OBJECTIVE
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.


CONCLUSIONS
Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.
Department of Paediatric Surgery, Royal London Hospital, London, UK.
Tariq
T
Burki
Liina
L
Kiho
Irene
I
Scheimberg
Simon
S
Phelps
Devesh
D
Misra
Harry
H
Ward
Isabel
I
Colmenero
D002467:Cell Nucleus / Q000473:pathology; D005260:Female; D045888:Ganglion Cysts / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000473:pathology*; D008297:Male; D008470:Meconium; D012007:Rectum / Q000473:pathology*; D012189:Retrospective Studies; D054047:Surgical Stomas
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