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Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review
Sarah Ullrich1, Kelly Austin2, Jeffrey R Avansino3
1Colorectal Center, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, USA.
Insights
Delayed diagnosis of Hirschsprung disease (HD) does not affect short-term surgical outcomes. However, later diagnosis increases the need for fecal diversion and interventions for constipation or incontinence after pull-through surgery.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Outcomes Research
Background:
- Hirschsprung disease (HD) is a rare congenital disorder causing intestinal obstruction in children.
- Diagnosis typically occurs neonatally, followed by pull-through (PT) surgery.
- Optimal management and outcomes for delayed HD diagnosis remain unclear.
Purpose of the Study:
- To evaluate the impact of age at diagnosis on post-operative outcomes in children with Hirschsprung disease.
- To compare complication rates, need for revision surgery, and long-term functional outcomes based on diagnostic timing.
Main Methods:
- Multi-center retrospective review of 679 pediatric patients with HD.
- Stratification by age at diagnosis: neonates (<29 days), infants (29 days-12 months), toddlers (1-5 years), and children (>5 years).
Main Results:
- Age at diagnosis did not influence 30-day complication rates or need for PT revision.
- Older age at diagnosis correlated with increased fecal diversion post-PT (28% in children vs. 10% in neonates).
- Delayed diagnosis was associated with higher rates of interventions for constipation/incontinence postoperatively.
Conclusions:
- Delayed diagnosis of Hirschsprung disease does not adversely affect immediate post-operative outcomes or revision rates.
- However, delayed diagnosis is linked to a greater need for fecal diversion and subsequent interventions for bowel dysfunction.
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.
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