Pull-through, diversion, or irrigation? Management of neonatal Hirschsprung disease in U.S. Children's hospitals.

Abstract

Purpose: Both neonatal pull-through and staged repair after rectal irrigations have become accepted strategies for Hirschsprung disease (HD) in newborns. Staged pull-through may be associated with better continence, although its impact on the incidence of Hirschsprung associated enterocolitis (HAEC) is not clear. We assessed variations in the management of neonatal HD and evaluated outcomes after neonatal versus staged pull-through.

Methods: The Pediatric Health Information Systems (PHIS) database was used to identify patients diagnosed with HD at less than 30 days of age at U.S. children's hospitals between 2017 and 2024 who underwent pull-through by 180 days. Operations performed within 10 days of diagnostic rectal biopsy were classified as primary pull-through procedures. Patients who underwent pull-through beyond that and had an enterostomy or not comprised the comparison groups. The incidence and severity of HAEC and incontinence was determined.

Results: Inclusion criteria were met in 662 patients, 44% of whom had a neonatal pull-through. Individual hospitals reported neonatal pull-throughs in 0-79% of cases. Patients managed with a staged pull-through without diversion had a similar incidence of HAEC and antibiotic duration as those who had a neonatal pull-through. Patients initially managed with diversion had more frequent and severe HAEC.

Conclusion: There was substantial variation among hospitals in the frequency of neonatal pull-through and diversion for HD. Staged repair without diversion was associated with a similar overall incidence of enterocolitis as neonatal pull-through. Given the continence benefits established in prior studies, staged pull-through may represent a preferable strategy for neonates with short-segment HD.

Type of study: Retrospective observational cohort study.

Level of evidence: III.