Moyamoya disease is a cerebrovascular disease of unknown etiology, which is characterized by bilateral stenosis or occlusion at terminal portion of internal carotid artery and at proximal portion of anterior cerebral artery and/or middle cerebral artery and abnormal vascular network in the vicinity of the arterial occlusions. It occurs frequently in Asian countries, particularly in Korea and Japan, but is rare in Western countries. To establish the etiology of moyamoya disease, much about the pathology from autopsies, factors involved in its pathogenesis, and its genetics have been studied. It may occur at any age from childhood to adulthood and in general, initial manifestation is cerebral ischemic symptoms in children and intracranial hemorrhage symptoms in adults. Because it progress and cause recurrent stroke, early diagnosis and proper management has been recognized. Cerebral angiography is essential for definitive diagnosis and treatment plan. Magnetic resonance imaging/magnetic resonance angiography is useful for diagnosis and follow-up tools after revascularization. Evaluation of the cerebral hemodynamics by single photon emission computed tomography and positron emission tomography is useful for diagnosis and assessment of the severity of cerebral ischemia in moyamoya patients. Surgical revascularization is effective for moyamoya disease manifesting as ischemic symptoms, to prevent further ischemia and infarction. In hemorrhagic type moyamoya disease, revascularization can be considered. Direct bypass, indirect synangiosis and combined methods are used. Outcomes of revascularization are excellent in preventing transient ischemic attacks in most patients.
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, Jun Hyeok Song Moyamoya disease is a cerebrovascular disease that features stenosis or occlusion at the distal internal carotid artery and proximal portion of the anterior and middle cerebal arteries. Before reversible changes occur to the brain revascularization surgery for patients of moyamoya disease is believed to prevent cerebral ischemic attacks by increasing and improving cerebral blood flow. The authors present the experiences of surgery for patents with ischemic or hemorrhage.
Revascularization surgeries were performed in 10 patents with episode of ischemic, seizure and hemorrhage between 1994 and 1999. There were 3 male and 7 female, aged 5 to 54(mean32.4 years) and 6 patients with ischemic, 3 patients with hemorrhage and 1 patient with seizure. Procedure of EDAS was performed in 4 patients with ischemic and 1 patient with hemorrhage. EMS was performed in 3 patients with hemorrhage and 1 patient with seizure. Combined EDAS and EMS was performed in 1 patients with ischemic. One superior cerebellar artery aneurysm was demonstrated and clipped and pseudoaneurysms was obseved in one patient. The authors studies the relationship between efficasy and outcome of surgical treatments.
All patients except one patient who died were followed up >3 years, from onset untill 1999. Improved and no recurred of symptoms and signs were observed in all patients who underwent EDAS procedure. Rebleeding was observed in 1 among 4 patients who underwent EMS procedure and the patient died of rebleeding 1 patient who underwent combined procedures EDAS and EMS resulted in improved and no recurrence of ischemic symptom. 1 superior cerebellar aneurysmal clipping was performed successfully. The pseudaneurysms arising dilating perforating moyamoya vessels in patient with hemorrhage were source of the hemorrhage and rebleeding.
The surgically treated patient seems to have good results, but long term study of large numbers of patient needs further investigation. Although the procedure of EDAS and EMS were successful in producing effective indirect arterial anastomoses and improvement in symptoms, superficial temporal artery middle cerebral artery(STA-MCA) anastomosis combined with indirect arterial anastomotic procedures were recommended as the appropriate surgical treatment for refracting cases of moyamoya disease.
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