ENCEPHALODUROARTERIOSYNANGIOSIS (EDAS) FOR THE TREATMENT OF CHILDHOOD MOYAMOYA DISEASE

Moyamoya disease is defined by the angiographic demonstration of stenosis or occlusion of the vessels of the anterior circulation at the base of the brain and the concomitant development of collateral blood supply. Untreated, the disease is often clinically progressive, resulting in significant neurologic sequelae. Encephaloduroarteriosynangiosis (EDAS), which involves the transposition of a segment of a scalp artery onto the surface of the brain, is a surgical treatment aimed at improving collateral blood flow. Six children underwent 8 EDAS procedures and were followed from 6 months to 9 years after surgery. No patient experienced further deterioration in neurologic status. Postoperative angiography demonstrated cerebral revascularization from the donor scalp artery on 3 of the 6 EDASs that were studied. The 2 patients who did not revascularize after EDAS demonstrated angiographic regression of their disease. The data suggest that EDAS is a safe procedure for the treatment of childhood moyamoya disease. Given the potential severity of the sequelae, early operative intervention is recommended in all children with this disease.