O2150: Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes?

Background: Hirschsprung disease is a common cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull through soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion is not known.

Methods: A multicenter retrospective review of children with Hirschsprung Disease was performed at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). Children were stratified by age at diagnosis (neonates < 29 days, infants 29 days – 12 months, and children > 12 months). The primary outcomes were post-operative complications within 30 days, need for fecal diversion post pull through, need for pull through revision and need for intervention for constipation or incontinence. Univariate analysis using Pearson’s Chi squared test was performed to evaluate for associations between age at diagnosis and patient-level factors.

Results: A total of 679 patients with Hirschsprung Disease from 14 sites were identified and included in our analysis. The majority of patients (n = 466, 69%) were diagnosed in the neonatal period, with 16% (n = 111) diagnosed as an infant and 15% (n = 102) were diagnosed as a child. There was no association with age at diagnosis and sex, race, ethnicity or transition zone (Table 1). Patients diagnosed as infants (n = 10, 9%) or children (n = 15, 15%) were more likely to have an associated gastrointestinal anomaly than those diagnosed as neonates (n = 33, 7%, P = 0.049, Table 1). Age at diagnosis was not associated with differences in 30 day complication rates or need for pull through revision (Table 2). Patients diagnosed at an older age were more likely to need fecal diversion post pull through (neonates n = 48, 10%, infants n = 13, 11%, children n = 27, 27%, P < 0.001). Older age at diagnosis was also associated with a greater likelihood of needing intervention for constipation or incontinence post pull through (neonates n = 263, 56%, infants n = 69, 62%, children n = 77, 76%, P = 0.002).

Conclusion: Delayed diagnosis of Hirschsprung Disease does not impact early post-operative outcomes or need for revision surgery but is associated with increased need for fecal diversion post pull through and bowel management for constipation or incontinence.