Moyamoya Syndrome: Pial Synangiosis

  • Edward R. Smith
  • R. Michael Scott


Moyamoya syndrome is an arteriopathy characterized by progressive stenosis at the apices of the intracranial internal carotid arteries associated with cerebral ischemia. As the internal carotids undergo this reduction in flow, the brain compensates through the development of smaller collateral vessels. This alternative blood supply provides circulation to the region formerly supplied by the internal carotids, and this fine network of vessels arises from the carotid apex, the leptomeninges, and branches of the external carotid artery supplying the dura and skull base. Although usually limited to the anterior circulation, there are rare cases where the process encompasses the posterior circulation as well, including the basilar and posterior cerebral arteries. The appearance of this collateral network on angiogram has been compared to a puffof smoke; in Japanese, “moyamoya.”


Middle Cerebral Artery Down Syndrome Moyamoya Disease Superficial Temporal Artery Acute Chest Syndrome 
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  1. 1.
    Scott RM, Smith ER (2009) Moyamoya disease and moyamoya syndrome. N Engl J Med. doi: 360/12/1226 [pii] 10.1056/NEJMra0804622Google Scholar
  2. 2.
    Smith ER, Scott RM (2008) Progression of disease in unilateral moyamoya syndrome. Neurosurg Focus 24:E17PubMedCrossRefGoogle Scholar
  3. 3.
    Hallemeier CL, Rich KM, Grubb RL Jr et al (2006) Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 37:1490–1496PubMedCrossRefGoogle Scholar
  4. 4.
    Scott RM, Smith JL, Robertson RL et al (2004) Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg Spine 100:142–149Google Scholar
  5. 5.
    Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299PubMedCrossRefGoogle Scholar
  6. 6.
    Imaizumi T, Hayashi K, Saito K et al (1998) Long-term outcomes of pediatric moyamoya disease monitored to adulthood. Pediatr Neurol 18:321–325PubMedCrossRefGoogle Scholar
  7. 7.
    Maki Y, Enomoto T (1988) Moyamoya disease. Childs Nerv Syst 4:204–212PubMedCrossRefGoogle Scholar
  8. 8.
    Fukuyama Y, Umezu R (1985) Clinical and cerebral angiographic evolutions of idiopathic progressive occlusive disease of the circle of Willis (“moyamoya” disease) in children. Brain Dev 7:21–37PubMedCrossRefGoogle Scholar
  9. 9.
    Kuroda S, Ishikawa T, Houkin K et al (2005) Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 36:2148–2153PubMedCrossRefGoogle Scholar
  10. 10.
    Fung LW, Thompson D, Ganesan V (2005) Revascularisation surgery for paediatric moyamoya: a review of the literature. Childs Nerv Syst 21:358–364PubMedCrossRefGoogle Scholar
  11. 11.
    Ohaegbulam C, Magge S, Scott RM (2001) Moyamoya syndrome. In: McLone DG (ed) Pediatric neurosurgery: surgery of the developing nervous system. Saunders, New YorkGoogle Scholar
  12. 12.
    Scott RM, Smith JL, Robertson RL et al (2004) Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg 100:142–149PubMedGoogle Scholar
  13. 13.
    Choi JU, Kim DS, Kim EY et al (1997) Natural history of moyamoya disease: comparison of activity of daily living in surgery and non surgery groups. Clin Neurol Neurosurg 99 Suppl 2:S11–18PubMedCrossRefGoogle Scholar
  14. 14.
    Lin Y, Yoshiko K, Negoro T et al (200) Cerebral oxygenation state in childhood moyamoya disease: a near-infrared spectroscopy study. Pediatr Neurol 22:365–369CrossRefGoogle Scholar
  15. 15.
    Fujiwara J, Nakahara S, Enomoto T et al (1996) The effectiveness of O2 administration for transient ischemic attacks in moyamoya disease in children. Childs Nerv Syst 12:69–75PubMedCrossRefGoogle Scholar
  16. 16.
    Nomura S, Kashiwagi S, Uetsuka S et al (2001) Perioperative management protocols for children with moyamoya disease. Childs Nerv Syst 17:270–274PubMedCrossRefGoogle Scholar
  17. 17.
    Jea A, Smith ER, Robertson R (2005) Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization. Pediatrics 116:e694–701PubMedCrossRefGoogle Scholar
  18. 18.
    Smith ER, McClain CD, Heeney M et al (2009) Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome. Neurosurg Focus, doi:10.3171/2009.01.FOCUS08307Google Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  1. 1.Department of NeurosurgeryChildren's Hospital, Harvard Medical SchoolBostonUSA

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