ID #740 Clinical Outcomes of Endoscopic Third Ventriculostomy for Hydrocephalus of Pediatric Diffuse Intrinsic Pontine Glioma Patients
Yuta Koketsu, Shoichi Deguchi, Junya Yamaguchi, Kazuhito Takeuchi, Yuichi Nagata, Yoshiki Shiba, Kosuke Aoki, Fumiharu Ohka, Ryuta SaitoAbstract
Objective
To evaluate clinical outcomes of pediatric cases with diffuse intrinsic pontine glioma (DIPG) treated at our institution and to analyze significance of endoscopic third ventriculostomy (ETV) in patients who developed hydrocephalus during clinical course.
Background
DIPG is a highly aggressive pediatric brain tumor with extremely poor prognosis. Previous studies reported that 15–60% of DIPG patients exhibit hydrocephalus within approximately 5 months after diagnosis, often further compromising quality of life. We aggressively perform endoscopic ETV for DIPG cases exhibiting suspected obstructive hydrocephalus based on MRI images.
Methods
We retrospectively reviewed the medical records of 27 consecutive pediatric patients with DIPG treated at our institution between January 2010 and December 2025. Demographic and clinical data, imaging findings, treatment details (including radiotherapy, biopsy, and CSF diversion), and outcomes were analyzed.
Results
The median age at diagnosis was 6 years (range, 2–13 years), and 8 patients (30%) were male. Stereotactic biopsy was performed in 3 patients (11%). All patients received initial radiotherapy with a planned dose of 54 Gy in 30 fractions, which was discontinued in 1 patient due to intratumoral hemorrhage. The median progression-free survival and overall survival were 5.6 and 11.7 months, respectively. Ventricular enlargement was found in 17 patients (68%) during the clinical course. Among them, we performed endoscopic ETV for 9 patients who developed symptoms associated with acute increasement of intracranial pressure (headache and vomiting in 6, impaired consciousness in 3) without significant ETV-related complications. All 9 patients showed rapid postoperative improvement of symptom. Among these 9 patients, for 6 patients (67%), ETV was performed within 3 months after initial diagnosis. No patients required subsequent permanent shunt placement. The median overall survival after ETV was 6.9 months.
Conclusions
The prognosis of DIPG remains dismal in the modern treatment era. For patients who developed ventricular enlargement at an early phase, ETV might be a safe and effective option for preservation of favorable quality of life during the remaining clinical course by preventing critical intracranial hypertension.