Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes?

Part of Pediatric Colorectal and Pelvic Reconstruction 15 items

Infographic · Aug 2024 · 1 min read

In brief

Multi-center study of 679 Hirschsprung patients examining whether delayed diagnosis affects surgical outcomes. Findings show delayed diagnosis doesn't impact revision rates but increases need for post-operative fecal diversion, likely due to anastomotic leaks in older children.

Delayed Hirschsprung diagnosis beyond neonatal period does not increase 30-day complications or revision surgery rates after pull-through.
Children diagnosed after age 1 year have 2-3× higher rates of requiring fecal diversion post-operatively (26-28% vs 10-12% in neonates/infants).
Older age at diagnosis correlates with increased need for interventions addressing postoperative constipation or incontinence (78% in toddlers vs 56% in neonates).
Most Hirschsprung patients (69%) are diagnosed in the neonatal period, but delayed presentations occur and require tailored management expectations.

Written by the GCMD Library team from the infographic.

A three-column comparison layout with medical illustrations of esophageal procedures. Each column contains anatomical diagrams in blue and pink tones showing the esophagus and surgical technique, accompanied by statistical data boxes below. The left column shows balloon dilation, the middle shows Heller myotomy, and the right shows POEM procedure.

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, Michael D Rollins, Payam Saadai, K Elizabeth Speck, Richard J Wood, Kathleen van Leeuwen, Jason S Frischer; Pediatric Colorectal and Pelvic Learning Consortium

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.

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