The neurodevelopmental outcome after endovascular treatment of vein of Galen malformation (VOGM) in 27 patients seen between 1983 and 2002 was assessed by chart review and parental questionnaires at the University of California, San Francisco. The presentation with congestive heart failure (CHF; 16/27) or hydrocephalus (8/27) was prenatal (diagnosed by ultrasound) in 5, neonatal in 16, and post-neonatal in 6. Patients with CHF presented either prenatally or neonatally, 4 died acutely, 6 were significantly delayed, and 6 had no or minor developmental delay. Of 5 presenting perinatally without CHF, all survived, 2 were significantly delayed, and 3 had no delay. Of 6 presenting after the neonatal period, all survived and only 1 was delayed. Those with choroidal VOGM by angiography had a worse prognosis (3/13 died; 5/13 delayed) than those with mural VOGM (2/10 had significant delay; none died). Of all cases in the series, 52% (61% of survivors) had no or minor delay. The features associated with the worst outcome were perinatal presentation, congestive heart failure, and choroidal angioarchitecture. [1]

COMMENT. The majority of infants with vein of Galen malformation who are selected for endovascular treatment have a favorable neurodevelopmental outcome. Neonatal presentation, congestive heart failure, and choroidal as opposed to mural angioarchitecture are features carrying the worst prognosis. The overall mortality is 15%. The majority of survivors have a good outcome with 52% having no delay and an additional 9% having only mild delay in neurodevelopment.