J Korean Med Assoc.  2007 Dec;50(12):1109-1118. 10.5124/jkma.2007.50.12.1109.

Update of Moyamoya Disease

• Lee JY
• Kim SK

• KMID: 2184959
• DOI: http://doi.org/10.5124/jkma.2007.50.12.1109

Abstract

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.

Keyword

Children; Moyamoya disease; Outcome; Revascularization

MeSH Terms

Adult
Angiography
Autopsy
Carotid Artery, Internal
Cerebral Angiography
Cerebral Hemorrhage
Cerebral Infarction
Child
Constriction, Pathologic
Diagnosis
Far East
Genetics
Hemodynamics
Humans
Infarction
Ischemia
Ischemic Attack, Transient
Japan
Korea
Magnetic Resonance Imaging
Moyamoya Disease*
Pathology

Figure

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  Figure 1 An axial T2-weighted MR image shows diminished flow voids in the internal carotid and middle cerebral arteries (A) and huge cortical infarction in the right hemisphere and left frontal lobe (B). An axial T1-weighted MR image demonstrates punctuate and curvilinear flow voids of the hypertrophied moyamoya collateral in the basal ganglia (C). An axial T1-weighted MR image with gadolinium enhancement reveals prominent leptomeningeal enhancement in the right hemisphere (D).

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  Figure 2 Rest (A) and acetazolamide (B) SPECT images show decreased perfusion with disturbed vasoreactivity to the acetazolamide injection in the right hemisphere.

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  Figure 3 A perfusion MRI demonstrates an increased rCBV (A) and delayed TTP (B) in the right hemisphere.

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  Figure 4 A right carotid angiography (A: AP view, B: lateral view) shows stenotic change in the terminal portion of the internal carotid artery, total occlusion in the middle cerebral artery and stenotic change in the proximal anterior cerebral artery. A left carotid angiography (C: AP view, D: lateral view) reveals stenotic change in the terminal portion of the internal carotid artery and the middle cerebral artery, and total occlusion in the anterior cerebral artery.

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  Figure 5 Operative illustrations of EDAS operation. A) The skin incision was made along the course of the right STA. B) The STA-galeal flap laid on the exposed cortex was sutured to the incised edge of the dura mater after dissection of the arachnoid membrane (Modified from (33) with the permission from Ilchokak).

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  Figure 6 Operative illustrations of bifrontal EG(P)S. A) S-shaped scalp incision was made 2 cm anterior to the coronal suture. B) The prepared galeo (-periosteal) flap was inserted into the cerebral cortex and sutured to the dura (Modified from (35) with the permission from Ilchokak).

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