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Shunt Complications in Syndromic versus Non-Syndromic Pediatric Hydrocephalus: A Propensity-Matched Multicenter
Barbara Buccilli1,2, Amna Hussein3,4, Khaled M Taghlabi3,4
1Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York, USA, doctor.b.buccilli@gmail.com.
Insights
Pediatric hydrocephalus patients with congenital syndromes face significantly higher risks for shunt reprogramming, revision, removal, and dysfunction compared to those without syndromes. This highlights the need for tailored surveillance and care strategies for these high-risk individuals.
Area of Science:
- Pediatric Neurosurgery
- Medical Genetics
- Biostatistics
Background:
- Congenital malformation syndromes can complicate pediatric hydrocephalus management by altering cerebrospinal fluid (CSF) dynamics.
- Understanding shunt-related complication rates in syndromic pediatric hydrocephalus is crucial for optimizing surgical outcomes.
Purpose of the Study:
- To compare shunt placement, reprogramming, revision, removal, and dysfunction rates in pediatric hydrocephalus patients with and without congenital syndromes.
- To identify specific congenital syndromes associated with increased shunt-related complications.
Main Methods:
- Retrospective analysis of pediatric hydrocephalus patients using the TriNetX database.
- Two cohorts were established: patients with congenital syndromes (N=17,617) and matched controls without syndromes (N=17,617).
- Propensity score matching was employed, and outcomes were assessed at 1-year follow-up.
Main Results:
- Shunt placement rates were similar between syndromic and non-syndromic cohorts.
- Syndromic patients exhibited significantly higher rates of shunt reprogramming (OR: 2.52), revision (OR: 3.03), removal (OR: 2.24), and dysfunction (OR: 2.52).
- Intervention-free survival was lower in syndromic patients, with specific syndromes like Arnold-Chiari and encephalocele showing shunt dysfunction rates exceeding 30%.
Conclusions:
- Pediatric hydrocephalus associated with congenital syndromes carries a substantially higher risk of shunt-related complications.
- These findings underscore the necessity for enhanced clinical surveillance and individualized care plans for syndromic pediatric hydrocephalus patients.
- Identifying high-risk phenotypes is essential for improving management strategies and patient outcomes.
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.
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