頭蓋内出血を伴う“もやもや”病

概要

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Seven cases of“Moyamoya”disease manifested with intracranial hemorrhage were presented, 2 males and 4 females, ranging 14 to 49 years old. Five of 6 cases initiated with hemorrhage, one once experienced with an old episode of TIA in the childhood. On angiogram aneurysm was demonstrated in moyamoya networks in two cases, one at the basilar top and the other in the periventricular region, and it was presumably responsible for hemorrhagic site. On CT scan of 5 cases, two different sites of hematoma, the basal ganglia and periventricular area favoring the trigone of the lateral ventricle, were characterized. According to the severity of the hemorrhage, 3 cases were treated conservatively and 4 cases surgically. Two of 4 cases required emergent surgery, ventricular drainage and hematoma evacuation respectively. After the acute stage, 2 months after the onset, STA-MCA bypass operation were performed in order to reduce the chance of repeated hemorrhage in the near future as well as to prevent ischemic problem. As long as followed-up period of 6 months to 7 years, prognosis was evaluated favorable in the most without repeated hemorrhage nor ischemic episode. Even in the hemorrhagic cases of“Moyamoya”disease the bypass operation will be available in terms of reducing the possibility of repeated hemorrhage, because of diminishing the networks, main sources of the hemorrhage, after the revasculization procedures.