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Pull-through, diversion, or irrigation? Management of neonatal Hirschsprung disease in U.S. Children's hospitals
Simone Carlson Hyman1, Sophia M V Schermerhorn2, Romeo Ignacio1
1Rady Children's Hospital, 3020 Children's Way, MC 5136, San Diego, CA, 92123, USA; University of California San Diego School of Medicine, Department of Surgery, 9500 Gilman Drive, La Jolla, CA 92093, USA.
Insights
Staged pull-through repair for Hirschsprung disease (HD) without diversion shows similar enterocolitis rates as neonatal pull-through. This approach may offer better continence for newborns with short-segment HD.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Gastroenterology
Background:
- Hirschsprung disease (HD) management in neonates involves neonatal pull-through or staged repair.
- The impact of staged pull-through on Hirschsprung-associated enterocolitis (HAEC) requires further clarification.
- Continence outcomes may favor staged pull-through, but comparative HAEC data is limited.
Purpose of the Study:
- To assess variations in neonatal HD management strategies.
- To compare outcomes, specifically HAEC incidence and severity, between neonatal and staged pull-through procedures.
- To evaluate the influence of diversion in staged repairs.
Main Methods:
- Retrospective analysis of the Pediatric Health Information Systems (PHIS) database (2017-2024).
- Inclusion of neonates (<30 days) diagnosed with HD and undergoing pull-through by 180 days.
- Classification of procedures into neonatal pull-through, staged pull-through with diversion, and staged pull-through without diversion.
Main Results:
- 662 patients met inclusion criteria; 44% underwent neonatal pull-through.
- Hospital variation in neonatal pull-through frequency (0-79%) was substantial.
- Staged pull-through without diversion showed similar HAEC incidence and antibiotic duration compared to neonatal pull-through.
- Diversion in staged repairs was linked to more frequent and severe HAEC.
Conclusions:
- Significant hospital-level variation exists in neonatal HD management, including pull-through timing and diversion use.
- Staged repair without diversion presents comparable enterocolitis rates to immediate neonatal pull-through.
- Considering established continence benefits, staged pull-through without diversion may be a preferred strategy for selected neonates with short-segment HD.
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.
