Moyamoya disease associated with hemophilia A. Case report.

A 10-year-old boy who had been diagnosed as having hemophilia A presented with episodes of transient ischemic attack. Cerebral angiography showed occlusions of the bilateral anterior cerebral arteries and the right middle cerebral artery with the development of an abnormal vascular network. The patient was diagnosed as having moyamoya disease associated with hemophilia A and subsequently underwent multiple burr hole surgery for revascularization under sufficient factor VIII supplementation. He remained asymptomatic after surgery, and follow-up cerebral angiography 5 months after the operation demonstrated significant neovascularization through the burr holes. Multiple burr hole surgery is proposed as a method of choice for the treatment of moyamoya disease associated with a bleeding tendency. Copyright 2002 S. Karger AG, Basel