Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium
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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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Does delayed diagnosis of Hirschrung disease affect postoperative and functional outcomes? I'm Alex Halpern, a research fellow from Children's National, and this is an article that you should know. The pediatric Colorectal and Pelvic Learning Consortium conducted a multi-center retrospective review from 2017 to 2023 trying to answer this question. They included 679 patients with Hirschprung disease from 14 different sites. They found that an increased age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after initial pull through procedure. They also found that increased, A diagnosis was associated with an increased risk of constipation or incontinence requiring intervention postoperatively. They did not find an association between age at diagnosis and 30 day complication rate after initial pull through or need for pull through revision. So, it seems like delayed diagnosis of Hirschrung's disease does affect certain outcomes in these patients. Does this information change your practice at all? Let us know what you think in the comments below.