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

Creator(s)

Sarah Ullrich
Kelly Austin
Jeffrey R. Avansino
Andrea Badillo
Casey M. Calkins
Rachel C. Crady
Megan M. Durham
Megan K. Fuller
Ankur Rana
Ron W. Reeder
Rebecca M. Rentea, Children's Mercy HospitalFollow
Michael D. Rollins
Payam Saadai
K Elizabeth Speck
Richard J. Wood
Kathleen van Leeuwen
Jason S. Frischer
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

Recommended Citation

Ullrich S, Austin K, Avansino JR, et al. Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium. J Pediatr Surg. 2024;59(7):1250-1255. doi:10.1016/j.jpedsurg.2024.03.034