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Published on: April 26, 2019

Long-term continence in patients with Hirschsprung's disease and Down syndrome

Anthony G Catto-Smith1, Misel Trajanovska, Russell G Taylor

  • 1Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Parkville, Victoria, Australia. tony.cattosmith@rch.org.au

Journal of Gastroenterology and Hepatology
|May 9, 2006
PubMed

Insights

Children with Down syndrome and Hirschsprung's disease often experience persistent bowel dysfunction, including incontinence and diarrhea. However, symptoms like constipation and fecal incontinence tend to improve with age in these patients.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Genetics

Background:

  • Hirschsprung's disease (HSCR) is frequently diagnosed in children with Down syndrome (DS).
  • The long-term bowel function and continence outcomes for children with co-occurring DS and HSCR remain poorly understood.
  • This study investigates the natural history of bowel function in this specific pediatric population.

Purpose of the Study:

  • To determine the natural history of bowel function in children diagnosed with both Down syndrome and Hirschsprung's disease.
  • To assess the prevalence of continence issues and other bowel dysfunctions in this cohort.
  • To identify potential factors influencing bowel function over time.

Main Methods:

  • A retrospective study was conducted on patients diagnosed with both DS and HSCR.
  • Data was collected from medical records at the Royal Children's Hospital, Melbourne, between 1974 and 2001.
  • A structured questionnaire was used to gather information on bowel function and continence from eligible patients.

Main Results:

  • Fecal incontinence was reported by 87% of patients, with 40% experiencing diarrhea and perianal excoriation.
  • Persistent constipation was less common, affecting 20% of the cohort.
  • A significant improvement in bowel dysfunction, including sensation, constipation, diarrhea, and excoriation, was observed with increasing age (P < 0.05).
  • An unexpectedly high prevalence of adverse food reactions (90%), particularly to fruits and vegetables, was noted.

Conclusions:

  • Children with Down syndrome and Hirschsprung's disease commonly experience persistent bowel dysfunction.
  • Evidence suggests that bowel function, including continence, improves with age in this population.
  • A high incidence of adverse reactions to food warrants further investigation in this patient group.
Abstract

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