Abstract WP221: Vein of Galen Malformations: Twenty Years Later

Abstract

Introduction: Vein of Galen Malformations (VOGM) are congenital arteriovenous malformations which carry a mortality of 76.7% in untreated patients. Outcomes have improved over time with advances in endovascular embolization. As endovascular management of VOGM has begun to favor more aggressive treatment strategies, there is scarce literature examining long-term functional and neurological outcomes and whether patients may have benefited from a more or less aggressive approach to treatment. Methods: A single-center retrospective review identified all patients with VOGM over the age of 18 with complete clinical data from January 1982 to May 2005. Patients were stratified based on radiographic outcomes: cured before the age of 18 (“pediatric-cured group”), cured after the age of 18, or not cured. Patients cured after the age of 18 and patients who were never cured were grouped together (“adult-cured group”). Long-term functional outcomes were assessed using the modified Rankin Scale (mRS). Statistical analyses were conducted in R version 4.2.1. Results: Forty-three adult patients were identified: 26 in the pediatric-cured group and 17 in the adult-cured group. The median current age of the pediatric-cured group was less than the adult-cured group (21 vs. 28, p<0.01). On initial presentation, the groups did not differ in their clinical or radiographic symptoms, comorbidities, or developmental delays. No significant differences were present in treatment courses. The pediatric-cured group demonstrated lower risk of intraprocedural or postprocedural hemorrhagic complications (p=0.03). On long-term follow-up, adult-cured patients presented with worse mRS (p<0.01), a higher rate of neurological symptoms (p<0.01), persistent fine motor delays (p<0.03), persistent cognitive or learning deficits (p=0.04), and persistent hydrogenous disorder (p<0.01). Conclusion: This is the first study to analyze long-term outcomes of adult patients managed for VOGM during the pediatric period. Patients cured in the pediatric period demonstrated reduced risk of hemorrhagic complications and improved long-term functional and neurological outcomes. Pursuing early cure in patients with VOGM may provide long-term benefit and reduce risk in later treatment.