Shunt Complications in Syndromic versus Non-Syndromic Pediatric Hydrocephalus: A Propensity-Matched Multicenter Analysis of 35, 234 Patients

Abstract

Introduction: Pediatric hydrocephalus is associated with congenital malformation syndromes, which may alter cerebrospinal fluid dynamics and increase the complexity of surgical management. We aimed to compare rates of shunt placement, reprogramming, revision, removal, and dysfunction in pediatric patients with hydrocephalus, with and without congenital syndromes. Methods: We conducted a retrospective analysis using TriNetX, identifying pediatric patients with hydrocephalus. Cohort 1 included patients with congenital syndromes (N = 17, 617). Cohort 2 included matched patients without syndromes (N = 17, 617). Propensity score matching was performed across demographic and clinical variables. Outcomes included shunt placement, reprogramming, revision, removal, and dysfunction. Risk measures were calculated at 1-year follow-up. Results: Shunt placement was similar between cohorts. However, syndromic patients had significantly higher rates of reprogramming (10.0% vs. 4.2%; odds ratio [OR]: 2.52; p < 0.001), revision (9.3% vs. 3.3%; OR: 3.03; p < 0.001), removal (2.0% vs. 0.9%; OR: 2.24; p < 0.001), and dysfunction (23.5% vs. 10.9%; OR: 2.52; p < 0.001). Intervention-free survival was lower in syndromic patients for all outcomes except initial shunt placement. Across single congenital syndromes, patients with hydrocephalus demonstrated elevated rates of shunt-related complications compared to matched controls. In conditions like Arnold-Chiari and encephalocele, the rates of shunt dysfunction exceeded 30%, with OR above 3.0 and highly significant p values. Alport syndrome, arhinencephaly, and craniosynostosis showed elevated risks for reprogramming, revision, and dysfunction. Down syndrome demonstrated higher dysfunction rates (27.1% vs. 14.3%; OR: 2.22). Despite identical shunt placement rates, the downstream need for surgical intervention diverged sharply, highlighting high-risk phenotypes. Conclusion: Pediatric hydrocephalus associated with congenital syndromes carries a markedly higher risk of shunt-related complications. These findings support the need for clinical surveillance and individualized care strategies in this group.