Outcomes at five to eight years of age for children with Hirschsprung's disease

Benjamin Saul Raywood Allin1, Charles Opondo2, Timothy John Bradnock3

  • 1National Perinatal Epidemiology Unit, University of Oxford, Oxford, Oxfordshire, UK benjaminallin@doctors.org.uk.

Insights

This study reveals that many children with Hirschsprung disease (HD) face long-term issues like incontinence and low quality of life. Addressing these core outcomes is crucial for improving patient care.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Clinical Outcomes Research

Background:

  • Hirschsprung disease (HD) is a congenital condition affecting the large intestine.
  • Long-term outcomes in children with HD require comprehensive evaluation.

Purpose of the Study:

  • To describe the core outcomes of Hirschsprung disease (HD) in UK and Irish primary school-aged children.
  • To assess functional outcomes, reoperation rates, and quality of life in a UK-wide cohort.

Main Methods:

  • Prospective cohort study (2010-2012) involving 28 UK/Irish pediatric surgical centers.
  • Data collected from birth up to 5-8 years of age, including clinical outcomes and parent/clinician reports.
  • Utilized the NETS 1HD core outcomes framework.

Main Results:

  • Of 239 children, 13% had a stoma and 9% were incontinent of urine at 5-8 years.
  • 79% maintained bowel movements without intervention, but 63% experienced fecal incontinence.
  • 44% required unplanned reoperations, and many reported lower quality of life and psychological well-being scores.

Conclusions:

  • This study provides a realistic overview of population-level outcomes for HD in school-aged children.
  • High rates of incontinence, reoperations, and reduced quality of life highlight significant challenges.
  • Prioritizing management of bladder, bowel, and psychological issues is essential for affected children.
Abstract

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