KMID : 0614720070500121109
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Journal of Korean Medical Association 2007 Volume.50 No. 12 p.1109 ~ p.1118
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Update of Moyamoya Disease
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Lee Ji-Yeoun
Kim Seung-Ki
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Abstract
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Moyamoya disease is characterized by bilateral stenosis or occlusion of distal internal carotid
artery (ICA) bifurcation including its proximal branches and abnormal vascular network
(moyamoya vessel, MMV) in the vicinity of the arterial occlusions. It is the most common pediatric
cerebrovascular disease in Eastern Asia, particularly in Korea and Japan. The etiology is still
unknown, but much about the pathology from autopsies, factors involved in its pathogenesis, and
its genetics have been studied and reported. It may cause ischemic attacks or cerebral infarctions
in children and cerebral hemorrhage in adults. Because of its aggressive clinical course in very
young children, the need for early detection and treatment has been recognized. Magnetic
resonance imaging (MRI)/MR angiography (MRA), cerebral hemodynamic studies, and cerebral
angiography are used for the diagnosis. The treatment basically focuses on prevention of further
ischemia and infarction through revascularization. Technically, direct and indirect bypass methods
are used. The treatment strategy needs to be individualized in each patient. Outcomes of
revascularization procedures are excellent in preventing transient ischemic attacks (TIAs) in most
patients.
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KEYWORD
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Children, Moyamoya disease, Outcome, Revascularization
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