International Journal of Medical Sciences

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17 December 2018

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Int J Med Sci 2015; 12(7):566-575. doi:10.7150/ijms.11719


Research Progress of Moyamoya Disease in Children

Jianmin Piao, Wei Wu, Zhongxi Yang, Jinlu Yu

Department of Neurosurgery, First Hospital of Jilin University, Changchun, 130021, P.R. China


During the onset of Moyamoya disease (MMD), progressive occlusion occurs at the end of the intracranial internal carotid artery, and compensatory net-like abnormal vessels develop in the skull base, generating the corresponding clinical symptoms. MMD can affect both children and adults, but MMD in pediatric patients exhibits distinct clinical features, and the treatment prognoses are different from adult patients. Children are the group at highest risk for MMD. In children, the disease mainly manifests as ischemia, while bleeding is the primary symptom in adults. The pathogenesis of MMD in children is still unknown, and some factors are distinct from those in adults. MMD in children could result in progressive, irreversible nerve functional impairment, and an earlier the onset corresponds to a worse prognosis. Therefore, active treatment at an early stage is highly recommended. The treatment methods for MMD in children mainly include indirect and direct surgeries. Indirect surgeries mainly include multiple burr-hole surgery (MBHS), encephalomyosynangiosis (EMS), and encephaloduroarteriosynangiosis (EDAS); direct surgeries mainly include intra- and extracranial vascular reconstructions that primarily consist of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. Indirect surgery, as a treatment for MMD in children, has shown a certain level of efficacy. However, a standard treatment approach should combine both indirect and direct procedures. Compared to MMD in adults, the treatment and prognosis of MMD in children has higher clinical significance. If the treatment is adequate, a satisfactory outcome is often achieved.

Keywords: Children, Moyamoya disease, surgical treatment, prognosis

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How to cite this article:
Piao J, Wu W, Yang Z, Yu J. Research Progress of Moyamoya Disease in Children. Int J Med Sci 2015; 12(7):566-575. doi:10.7150/ijms.11719. Available from