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 ha s yet to be clearly established. Furthermore, it remains unclear as to whether or not bypass surgery can help prevent future intracranial he morrhaging in Moyamoya patients. In Japan, several treatment options, such as conservative, medical, and surgical intervention, have been em ployed for the treatment of hemorrhagic type of Moyamoya disease. In t his study, 282 hemispheres with hemorrhagic onset were analyzed based on a 1995 nationwide survey by the Research Committee on Spontaneous O cclusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare Japan to clarify the current status of treatment f or 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% we re medically treated. Ventricular drainage and/or hematoma removal was performed in 15.8%, and revascularization surgery in 38.3% of all hem orrhagic sides. Among the revascularization procedures used, 45.7% of the hemispheres undervent single indirect bypass surgery, such as EDAS while 22.2% of them received a direct (superficial temporal artery-mi ddle cerebral artery; STA-MCA) bypass surgery. A combination of direct and indirect bypass surgery or a combination of different kinds of in direct procedures comprised 32.1%. Forty-nine out of 282 hemorrhagic h emispheres demonstrated rebleeding. An intracranial hemorrhage occurre d in 15 hemispheres with ischemic onset even though nine of them had u ndergone bypass surgery prior to hemorrhaging. Nearly 18% of the patie nts with hemorrhagic type disease experienced rebleeding regardless of the treatment modalities. Based on these findings, there remains no c learly superior treatment plan for hemorrhagic Moyamoya disease to pre vent rebleeding at this time. However, the selection of patients, trea tment modalities, and the timing of the surgery might all play an impo rtant role in controlling rebleeding. The final outcome of the patient s are therefore mainly considered to correlate with the initial severi ty of the clinical features. (C) 1997 Elsevier Science B.V.