Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium.

Document Type

Article

Publication Date

7-2024

Identifier

DOI: 10.1016/j.jpedsurg.2024.03.034

Abstract

BACKGROUND: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown.

METHODS: A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonatesdays; infants 29 days-12 months; toddler 1 year-5 years and child >5 years).

RESULTS: 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001).

CONCLUSION: Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through.

LEVEL OF EVIDENCE: III.

Journal Title

Journal of pediatric surgery

Volume

59

Issue

7

First Page

1250

Last Page

1255

MeSH Keywords

Humans; Hirschsprung Disease; Retrospective Studies; Infant; Delayed Diagnosis; Child, Preschool; Female; Male; Infant, Newborn; Postoperative Complications; Treatment Outcome; Child; Reoperation

Keywords

Functional incontinence; Hirschsprung disease; Postoperative outcomes; Pull-through

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