409 Disseminated Pediatric Low-Grade Glioma and Hydrocephalus: A Multinational Consortium Analysis of Incidence and Mortality

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INTRODUCTION: Disseminated pediatric low-grade gliomas (DPLGG) are a rare subtype of an otherwise common tumor, characterized by leptomeningeal dissemination, with microtumors spreading throughout the CNS. The impact of this dissemination on cerebrospinal fluid (CSF) dynamics remains unexplored. We report the occurrence and impact of CSF diversion in pediatric patients with DPLGG. METHODS: A multicenter international, retrospective cohort study identified 261 children from 39 sites diagnosed with low-grade gliomas from 1988 to 2025. Patients were grouped by presence of hydrocephalus and subsequent CSF diversion procedure. They were further subdivided by modality including: ventriculoperitoneal shunting (VPS), endoscopic 3rd ventriculostomy (ETV), and endoscopic septostomy. Demographic, pathologic, radiographic, and treatment variables were collected. The primary outcomes were mortality and diversion failure. Group comparisons were conducted using independent sample t-tests and chi-squared analyses. Kaplan-Meier was used to assess survival and time-to-failure. RESULTS: One-hundred forty-five (55.6%) patients developed hydrocephalus and required CSF diversion. Pathologic diagnosis differed between groups (p = 0.02). Patterns of tumor dissemination did not significantly impact hydrocephalus development (p = 0.37), but timing of CSF diversion was impacted by dissemination pattern (p < .001). Kaplan-Meier survival analysis showed no difference in overall survival (log-rank = 0.95). However, hydrocephalus was associated with academic difficulties (p = .02) and concurrent endocrine disorders (p = .03). For initial diversion, thirteen patients (9.2%) underwent endoscopic third ventriculostomy (ETV), four received a septostomy, while 124 patients (87.9%) received ventriculoperitoneal shunts (VPS). Time to failure by modality showed no significant difference on Kaplan-Meier (log-rank test, p = 0.90) CONCLUSIONS: Hydrocephalus does not impact survival in DPLGG patients but is associated with decreased functional outcomes. Dissemination status impacts CSF diversion timing, but not hydrocephalus incidence. VPS is the predominant treatment, though high failure rates remain a challenge.

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