The impact of Down's syndrome on the immediate and long-term outcomes of children with Hirschsprung's disease

Pediatr Surg Int. 2006 Feb;22(2):179-81. doi: 10.1007/s00383-005-1617-0. Epub 2005 Dec 14.


Hirschsprung's disease (HD) in Down's syndrome (DS) patients is stated to have a worse outcome than HD alone. In our study we reviewed the immediate and long-term outcomes of these children and questioned whether DS should influence the operative management. Data were collected on all children with HD (including total colonic aganglionosis), between January 1990 and December 2000. They were divided into two groups based on the presence or absence of Trisomy 21 and compared retrospectively. In this time period we treated 173 children with HD; 17 of these had DS. Both the groups were comparable in their mean gestational age, birth weight and presentation except that the DS group had a significantly higher overall incidence of pre and/or postoperative enterocolitis. A tota1 of 164 children underwent a Swenson pull-through and 9 had a Soave's procedure. Follow-up ranged from 1 to 10 years. Continence assessed using the Wingspread scoring system in children over the age of 4 years showed no significant difference. Although children with both HD and DS are predisposed to complications and required a more cautious management, long-term outcome in terms of continence was not significantly worse than in HD alone. Thus the co-existence of DS should not influence the decision to offer these children and their parents the choice of definitive repair.

Publication types

  • Comparative Study

MeSH terms

  • Case-Control Studies
  • Child
  • Down Syndrome / complications*
  • Enterocolitis / etiology
  • Fecal Incontinence / etiology
  • Female
  • Hirschsprung Disease / complications*
  • Hirschsprung Disease / surgery*
  • Humans
  • Infant, Newborn
  • Male
  • Proctocolectomy, Restorative* / adverse effects
  • Retrospective Studies
  • Treatment Outcome