Continuing Education Column Update of Moyamoya Disease Ji Yeoun Lee, MDSeungKi Kim, MD Division of Pediatric Neurosurgery, Seoul National University College of Medicine Email : nsthomas@snu.ac.kr J Korean Med ssoc 2007; 50(12): 1109-1118 bstract Moyamoya disease is characterized by bilateral stenosis or occlusion of distal internal carotid artery (IC) 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 sia, 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. ecause 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 (MR), 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 (TIs) in most patients. Keywords : Children; Moyamoya disease; Outcome; Revascularization 1109
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Moyamoya Disease C D Figure 1. n axial T2weighted MR image shows diminished flow voids in the internal carotid and middle cerebral arteries () and huge cortical infarction in the right hemisphere and left frontal lobe (). n axial T1weighted MR image demonstrates punctuate and curvilinear flow voids of the hypertrophied moyamoya collateral in the basal ganglia (C). n axial T1weighted MR image with gadolinium enhancement reveals prominent leptomeningeal enhancement in the right hemisphere (D). 1111
Lee JY Kim SK Figure 2. Rest () and acetazolamide () SPECT images show decreased perfusion with disturbed vasoreactivity to the acetazolamide injection in the right hemisphere. Figure 3. perfusion MRI demonstrates an increased rcv () and delayed TTP () in the right hemisphere. 1112
Moyamoya Disease C D Figure 4. right carotid angiography (: P view, : 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. left carotid angiography (C: P 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. 1113
Lee JY Kim SK STfrontal branch Scalp incision Figure 5. Operative illustrations of EDS operation. ) The skin incision was made along the course of the right ST. ) The STgaleal 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). Scalp incision STparietal branch Coronal Suture Dura Left galeo (periosteal) flap Superior sagittal sinus STparietal branch Right galeo (periosteal) flap Figure 6. Operative illustrations of bifrontal EG(P)S. ) Sshaped scalp incision was made 2 cm anterior to the coronal suture. ) The prepared galeo (periosteal) flap was inserted into the cerebral cortex and sutured to the dura (Modified from (35) with the permission from Ilchokak). 1114
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Lee JY Kim SK 11. Suzuki J, Takaku. Cerebrovascular "moyamoya" disease. Disease showing abnormal netlike vessels in base of brain. rch Neurol 1969; 20: 288-299. 12. Natori Y, Ikezaki K, Matsushima T, Fukui M. 'ngiographic moyamoya' its definition, classification, and therapy. Clin Neurol Neurosurg 1997; 99(S2): S168-172. 13. Ikezaki K, Han DH, Kawano T, Kinukawa N, Fukui M. clinical comparison of definite moyamoya disease between South Korea and Japan. Stroke 1997; 28: 2513-2517. 14. Suzuki J, Kodama N. Moyamoya disease a review. Stroke 1983; 14: 104-109. 15. Nakashima H, Meguro T, Kawada S, Hirotsune N, Ohmoto T. Long--term results of surgically treated moyamoya disease. Clin Neurol Neurosurg 1997; 99(S2): S156-161. 16. Ikezaki K. Clinical manifestations: epidemiology, symptoms and signs, laboratory findings. In: Ikezaki K, Loftus C, eds. Moyamoya Disease. Rolling Meadows: merican ssociation of Neurological Surgeons 2001; 43-54. 17. 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Neurosurgery 2004; 54: 840-844; discussion 4-6. 17. Ogawa, Nakamura N, Yoshimoto T, Suzuki J. Cerebral blood flow in moyamoya disease. Part 2: utoregulation and CO2 response. cta Neurochir (Wien) 1990;105:107-111. 18. Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho K. Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg 2005; 103: 439-442. 19. Ikezaki K, Fukui M, Inamura T, Kinukawa N, Wakai K, Ono Y. The current status of the treatment for hemorrhagic type moyamoya disease based on a 1995 nationwide survey in Japan. Clin Neurol Neurosurg 1997; 99(S2): S183-186. 20. Saeki N, Nakazaki S, Kubota M, Yamaura, Hoshi S, Sunada S, Sunami K. Hemorrhagic type moyamoya disease. Clin Neurol Neurosurg 1997; 99(S2): S196-201. 21. Iwama T, Morimoto M, Hashimoto N, Goto Y, Todaka T, Sawada M. Mechanism of intracranial rebleeding in moyamoya disease. Clin Neurol Neurosurg 1997; 99(S2): S187-190. 22. 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Cerebrovascular response to hypercapnia in pediatric and adult patients with moyamoya disease. Stroke 1997; 28: 701-707. 27. Seol HJ, Wang KC, Kim SK, Lee CS, Lee DS, Kim IO, Cho K. Unilateral (probable) moyamoya disease: longterm followup of seven cases. Childs Nerv Syst 2006; 22: 145-150. 28. Okada Y, Shima T, Matsumura S, Nishida M, Yamada T, Okita 1116
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Lee JY Kim SK Peer Reviewer Commentary 1118