Moyamoya disease and syndrome.

Moyamoya disease was first described by Suzuki in 1963. The surgical management of Moyamoya disease began in the mid 1970s. At our institution we began operating on Moyamoya disease in 1979 and between 1979 and 1995 we have treated 30 children with Moyamoya disease and Moyamoya syndrome. Of these 21 children have an excellent outcome. Two children continued to have symptoms and both responded to an encephalo-myo-synangiosis (EMS) procedure, five children had a good outcome but are left with a significant neurologic deficit, one child remains in a poor state and one child had an excellent outcome but then died of his Fanconi's anemia 7 years after his encephalo-duro-arterio-synangiosis (EDAS) procedure.