The current status of the treatment for hemorrhagic type moyamoya disease based on a 1995 nationwide survey in Japan.

The optimal treatment regimen for hemorrhagic type Moyamoya disease has yet to be clearly established. Furthermore, it remains unclear as to whether or not bypass surgery can help prevent future intracranial hemorrhaging in Moyamoya patients. In Japan, several treatment options, such as conservative, medical, and surgical intervention, have been employed for the treatment of hemorrhagic type of Moyamoya disease. In this study, 282 hemispheres with hemorrhagic onset were analyzed based on a 1995 nationwide survey by the Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare Japan to clarify the current status of treatment for hemorrhagic type Moyamoya disease. Questionnaires were distributed to the departments of neurology, neurosurgery, and pediatrics all over Japan asking about both treatment and rebleeding. As a result, 12.5% of the affected hemispheres were treated conservatively while 32.3% were medically treated. Ventricular drainage and/or hematoma removal was performed in 15.8%, and revascularization surgery in 38.3% of all hemorrhagic sides. Among the revascularization procedures used, 45.7% of the hemispheres underwent single indirect bypass surgery, such as EDAS while 22.2% of them received a direct (superficial temporal artery--middle cerebral artery: STA-MCA) bypass surgery. A combination of direct and indirect bypass surgery or a combination of different kinds of indirect procedures comprised 32.1%. Forty-nine out of 282 hemorrhagic hemispheres demonstrated rebleeding. An intracranial hemorrhage occurred in 15 hemispheres with ischemic onset even though nine of them had undergone bypass surgery prior to hemorrhaging. Nearly 18% of the patients with hemorrhagic type disease experienced rebleeding regardless of the treatment modalities. Based on these findings, there remains no clearly superior treatment plan for hemorrhagic Moyamoya disease to prevent rebleeding at this time. However, the selection of patients, treatment modalities, and the timing of the surgery might all play an important role in controlling rebleeding. The final outcome of the patients are therefore mainly considered to correlate with the initial severity of the clinical features.