Long-Term Outcome in Children with Vein of Galen Aneurysmal Malformation

Purpose: To evaluate the long term clinical outcome of a series of francophone children with vein of Galen malformation taken in charge in a single institution.

Methods: Single center study of a consecutive series of children born between January 2004 and December 2009 (6–10 year-old at last clinical assessment). The neurological long term outcome was assessed with the Bicetre Outcome Score (BOS), the Rivermaid Post-Concussion Questionnaire (RPQ) and the modified Rankin Scale (mRS).

Results: Fifty–two patients including 5 fetuses and 47 children was included. The sex ratio m/f was 33%/62% (missing data for 3 fetuses). Five fetuses had a termination of pregnancy due to severe prenatal neurological presentation on fetal MRI. The mortality rate was 37% (14 patients) taking in consideration that 11 of them received withdrawal care because of diffuse cerebral lesions. Among the 33 patients alive at the time of the present study, 20 had a normal or subnormal psychomotor development and 13 a delayed psychomotor development.

Conclusion: Children with MAVG are at risk of psychomotor delay. However, in the present series, 45% have a good development. Eighteen percent of patients presented a severe psychomotor delay and disability. The high mortality rate (37%) was mainly peri-natal.