Objective: Describe the course and outcomes in a UK national cohort of neonates with vein of Galen malformation (VGM) identified before 28 days of life. //
Methods:
Neonates with angiographically confirmed vein of Galen malformation presenting to one of the two UK treatment centres (2006‐2016) were included; those surviving were invited to participate in neurocognitive assessment. Results in each domain were dichotomised into “good” and “poor” categories. Cross‐sectional and angiographic brain imaging studies were systematically interrogated. Logistic regression was used to explore potential outcome predictors. //
Results:
Of 85 children with neonatal vein of Galen malformation, 51 had survived. Thirty‐four participated in neurocognitive assessment. Outcomes were approximately evenly split between “good” and “poor” categories across all domains, namely neurological status, general cognition, neuromotor skills, adaptive behaviour, and emotional and behavioural development. Important predictors of poor cognitive outcome were initial Bicetre score </=12 and presence of brain injury, specifically white matter injury, on initial imaging; in multivariable analysis only Bicetre score </=12 remained significant. //
Interpretation:
Despite modern supportive and endovascular treatment, over a third of unselected newborns with vein of Galen malformation did not survive. Outcome was good in around half of survivors. The importance of white matter injury suggests that abnormalities of venous, as well as arterial, circulation are important in pathophysiology of brain injury
