Hydrocephalus in Patients With Diffuse Intrinsic Pontine Glioma: A Systematic Review of the Literature

Hydrocephalus is an underexplored complication in patients with diffuse intrinsic pontine glioma (DIPG). This systematic review aims to evaluate the incidence, diagnosis, management strategies, and clinical impact of hydrocephalus in DIPG. We systematically searched MEDLINE and EMBASE databases up to Week 22 of 2025 for studies reporting hydrocephalus in DIPG patients, including their diagnosis, treatment, and outcomes. Inclusion criteria targeted studies involving confirmed DIPG or brainstem gliomas (excluding focal or exophytic forms) and reporting relevant clinical data. Among 266 DIPG patients across seven studies, 100 (37.5%) developed hydrocephalus. CSF diversion was performed in 94 patients (61 with ventriculoperitoneal shunt, 33 with endoscopic third ventriculostomy). In five studies, CSF diversion was associated with improved survival when hydrocephalus occurred during tumor progression (OS: 2.7-16.6 months). Limited evidence supports a survival benefit for patients treated at diagnosis. Surgical intervention appeared safe, with low complication rates. Hydrocephalus significantly impacts the clinical course of DIPG, yet standardized diagnostic criteria and treatment guidelines are lacking. Surgical CSF diversion at progression offers potential clinical and survival benefits. Larger prospective studies are warranted to define optimal management strategies and to evaluate hydrocephalus treatment as a means to sustain eligibility for evolving therapeutic trials.