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Related Experiment Video

Updated: Jan 20, 2026

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Does Hirschsprung-Associated Enterocolitis Differ in Children With and Without Down Syndrome?

Devin R Halleran1, Hira Ahmad2, Elias Maloof2

  • 1Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio; Center for Surgical Outcomes Research, Department of Surgery, The Research Institute at Nationwide Children's Hospital, Columbus, Ohio.

The Journal of Surgical Research
|September 3, 2019
PubMed
Summary

Children with Down syndrome (DS) and Hirschsprung disease (HD) experience more severe Hirschsprung-associated enterocolitis (HAEC) episodes. Further research is needed to develop preventive strategies for this vulnerable pediatric population.

Keywords:
Down syndrome (DS)EnterocolitisHirschsprung disease (HD)Hirschsprung-associated enterocolitis (HAEC)OutcomesTrisomy 21 (T21)

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Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Genetics

Background:

  • Hirschsprung disease (HD) is a congenital condition affecting the large intestine.
  • Children with HD and Down syndrome (DS) have a higher incidence of Hirschsprung-associated enterocolitis (HAEC).
  • The severity of HAEC episodes in children with both HD and DS is not well understood.

Purpose of the Study:

  • To compare the severity of HAEC episodes in pediatric patients with HD, with and without DS.
  • To identify clinical characteristics and outcomes associated with HAEC in this patient population.

Main Methods:

  • Retrospective cohort study of 86 HAEC admissions between 2009 and 2017.
  • Patients with HD and HAEC were divided into two groups: with and without DS.
  • Demographics, clinical presentation, and outcomes were compared between the groups.

Main Results:

  • Patients with DS (12% of cohort) were more likely to present with tachycardia and hypotension.
  • DS patients experienced longer symptom duration before treatment and higher rates of ICU admission.
  • While antibiotic courses were longer for DS patients, overall length of stay was similar.

Conclusions:

  • Pediatric patients with HD and DS appear to experience more severe HAEC episodes.
  • Increased morbidity associated with HAEC in children with HD and DS warrants further investigation.
  • Future research should focus on developing preventive strategies for HAEC in this high-risk group.