Document Type

Article

Publication Date

8-2026

Identifier

DOI: 10.1016/j.jpedsurg.2026.163227

Abstract

INTRODUCTION: We evaluated whether Trisomy 21 (T21) affected bowel management use, ability to toilet train for stool, and rates of Hirschsprung-Associated Enterocolitis (HAEC) in children with Hirschsprung disease (HD). We hypothesized that T21 would be associated with increased rates of HAEC, bowel management program (BMP) use, and delayed toilet training for stool.

METHODS: Children with HD in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database who underwent primary pull-through were included and stratified by T21 status: HD only versus HD + T21. Children requiring a redo pull-through, initially managed with a stoma, or those who had inadequate follow-up were excluded. HAEC episodes, toilet-training status, and BMP data recorded during individual PCPLC sites' clinical visits were analyzed.

RESULTS: 339 children with HD and 48 with HD + T21 were included. No significant differences in patient demographics, age at diagnosis, transition zone, age/weight at pull-through, type of pull-through, or child opportunity index (COI) were identified. No significant differences in BMP or number of HAEC episodes were found. Children with HD + T21 were less likely to be toilet trained at their 4-7-year visit (22.2% vs. 52.6%, p = 0.002) and their 8-11-year visit (22.2% vs. 80.9%, p < 0.001). On multivariable regression, T21 independently predicted lower odds of toilet training for stool at age 4-7 years.

CONCLUSIONS: Children with HD and T21 have delayed toilet training but have similar bowel management needs and HAEC rates to children with HD only, suggesting that differences in continence outcomes are not explained by increased enterocolitis burden or greater bowel management intensity.

LEVEL OF EVIDENCE: Level 3 - Retrospective Comparative Study.

Journal Title

Journal of pediatric surgery

Volume

61

Issue

8

First Page

163227

Last Page

163227

MeSH Keywords

Humans; Hirschsprung Disease; Enterocolitis; Down Syndrome; Male; Female; Infant; Retrospective Studies; Toilet Training; Child, Preschool; Postoperative Complications; Follow-Up Studies

PubMed ID

42251887

Keywords

Bowel function; Bowel management; Enterocolitis; Hirschsprung disease; Trisomy 21

Comments

This is an open access article distributed under the terms of the Creative Commons CC-BY license, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Publisher's Link: https://www.jpedsurg.org/article/S0022-3468(26)00310-6/fulltext

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