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 Jul;59(7):1250-1255. doi: 10.1016/j.jpedsurg.2024.03.034. Epub 2024 Mar 18.

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 (neonates <29 days; 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.

Keywords: Functional incontinence; Hirschsprung disease; Postoperative outcomes; Pull-through.

Publication types

  • Multicenter Study

MeSH terms

  • Child
  • Child, Preschool
  • Delayed Diagnosis* / statistics & numerical data
  • Female
  • Hirschsprung Disease* / diagnosis
  • Hirschsprung Disease* / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Postoperative Complications* / diagnosis
  • Postoperative Complications* / epidemiology
  • Postoperative Complications* / etiology
  • Reoperation / statistics & numerical data
  • Retrospective Studies
  • Treatment Outcome