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

Topic overview

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.

Intended audience: Healthcare professionals and clinicians.