The effect of encephalo-myo-synangiosis on abnormal collateral vessels in childhood moyamoya disease.

Child patients with Moyamoya disease initially present with ischemic symptoms. However, the long-term risk of intracranial hemorrhage for childhood Moyamoya disease is unknown. Hemodynamic overload to the fragile collateral vessels has been considered to cause hemorrhage. We reviewed angiograms to evaluate the effect of encephalo-myo-synangiosis (EMS) on abnormally dilated collateral vessels in 13 child patients with Moyamoya disease. EMS was performed on 24 sides in 13 patients ranging from 5 to 14 years of age. Post-operative angiography (6-88 months after surgery) revealed good revascularizations through EMS (larger than one-third of the middle cerebral artery (MCA) distribution) in 18 sides (75%) and smaller revascularizations in 6 sides (25%). In cases with a good revascularization through EMS, reduction of the abnormal collateral vessels was observed not only in the basal Moyamoya vessels (94% of sides) but also in the medullary arteries derived from the choroidal arteries (62% of sides), which are considered to cause intraventricular hemorrhages in adult patients. It is suggested that EMS may reduce the hemodynamic load on dilated collateral vessels and, subsequently, the long-term risk of intracranial hemorrhage in childhood Moyamoya disease.