Endoscopic Third Ventriculostomy for Adjunctive Management of Hydrocephalus Associated With Previously Embolized Vein of Galen Malformations.

Abstract

Hydrocephalus is a comorbid condition in patients with vein of Galen malformations (VOGMs), and its management is complicated by venous hypertension, elevating surgical risk. Endoscopic third ventriculostomy (ETV) is commonly used for cerebrospinal fluid (CSF) diversion in children, but there are limited available data on its efficacy and safety for patients with VOGM. We aimed to review our experiences with ETV across 2 high-volume centers for VOGM patients with hydrocephalus. A two-center retrospective review identified all patients with VOGM who underwent ETV. Demographics, clinical presentation, procedural history, and outcomes were reviewed. ETV success scores were calculated. Twelve patients with VOGM underwent ETV; 66.7% was male, with a median age of 6 months. Seven (58.3%) were still undergoing VOGM embolization before ETV, and 4 had a history of hydrocephalus with acute-on-chronic exacerbation. Eight patients (66.7%) presented with obstructive hydrocephalus because of aqueductal stenosis (58.3%) and with intraventricular hemorrhage without CSF pathway stenosis (8.3%). Three (25.0%) required external ventricular drain placement or fontanelle taps pre-ETV, and choroid plexus cauterization was performed in 4 patients (33.3%). Technical success of ETV was achieved in all cases. One patient experienced venous bleeding at the ventriculostomy site. Postoperative complications occurred in 2 patients: one with transient truncal ataxia and the other with seizure activity, all of which resolved by discharge. At most recent follow-up, 10 patients (83.3%) have minimal residual arteriovenous shunting of their VOGM. The mean ETV success score was 60.8, and 11 patients (91.7%) experienced clinical improvement with ETV patency at the 6-month follow-up. One patient required conversion to a ventriculoperitoneal shunt because of lack of improvement at 6 months. This largest-to-date series on VOGM-associated hydrocephalus treated with ETV demonstrates high safety and efficacy across 2 independent high-volume centers. In patients with VOGM demonstrating clinical deterioration secondary to hydrocephalus, ETV with possible choroid plexus cauterization is a viable CSF diversion strategy to consider.