Neurointerventional Treatment of Vein of Galen Malformation (VGM): A Structured Review with a Proposal for the Comparison of Outcome Quality

Abstract

Background: Vein of Galen malformation (VGM) is a congenital intracranial vascular anomaly consisting of arteriovenous fistulas and/or malformations between various arterial feeders and the median prosencephalic vein of Markowski (MPV). Despite its rare occurrence, VGM is of particular clinical relevance, as the excessive intracranial shunt volume leads to high mortality without appropriate treatment. Methods: The objective of this article is to review the published data on neurointerventional treatment and compare outcome quality in the included studies. Eight studies were included and synthesized. One study was multicentric and the rest were retrospective monocentric (level 4 evidence studies according to the Oxford Centre for Evidence-based Medicine). Results: The total number of included patients was 480 and patient age ranged from 1 day to 18 years. Mild or severe heart failure, hydrocephalus, and other reasons led to the indication for neurointerventional treatment, which was performed in all studies in the form of embolization. Under consideration of the introduced semiquantitative multidimensional scoring system, the highest total score, i.e., the best outcome quality, was found for the study “Houston” 2002–2018 (19 points) and the study “Duisburg” 2001–2010 (19 points). Conclusions: Neurointerventional treatment represents the essential pillar in the interdisciplinary management of patients with VGM, although standardization is lacking—based on the results of the structured review. As complementary treatments, pediatric critical care is mandatory and includes medical hemodynamic stabilization.

Keywords

• vascular malformation
• Vein of Galen malformation
• endovascular treatment
• embolization
• pediatric critical care