Bony carotid canal hypoplasia in patients with moyamoya disease

Clinical article

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The natural history of moyamoya disease is not well known. We have observed that the bony carotid canal is hypoplastic in patients with adult onset moyamoya disease. Bony carotid canal development should represent internal carotid artery (ICA) development, and may stop with the beginning of ICA stenosis. The purpose of this study was to determine the onset of moyamoya disease by measuring the bony carotid canal.


The normal diameter of the bony carotid canal was evaluated on 4-mm thick bone window CT scans of the skull base in 60 Japanese patients aged 20–80 years, who had minor head trauma or headache considered to be unrelated to the skull base or arterial systems. The relationship between age and bony carotid canal development was assessed in a second group of 50 patients aged 0–19 years, including 10 under 2 years, using CT scans with the same parameters. The diameter of the bony carotid canal in 17 Japanese patients with moyamoya disease was measured.


The normal diameter in adults was 5.27 ± 0.62 mm (mean ± SD). The bony carotid canal developed rapidly before approximately 2 years of age. After fusion of the bony suture, the bony carotid canal developed slowly. The mean diameter of the bony carotid canal was 3.31 ± 0.44 mm in 11 adult patients with adult-onset moyamoya disease. According to the apparent curve of bony carotid canal development, ICA stenosis was assumed to start in early childhood.


Our findings suggest that most cases of Asian moyamoya disease may arise in childhood and that many Asian adult patients with moyamoya disease may develop occlusive vasculopathy in childhood.

Abbreviation used in this paper: ICA = internal carotid artery.
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Contributor Notes

Address correspondence to: Arata Watanabe, M.D., Ph.D., Department of Neurosurgery, Tsuru Municipal Hospital, 5-1-55, Tsuru, Tsuru City, Yamanashi 402-0056, Japan. email to:
  • 1

    Cali RLBerg RRama K: Bilateral internal carotid artery agenesis: a case study and review of the literature. Surgery 113:2272331993

    • Search Google Scholar
    • Export Citation
  • 2

    Hallemeier CLRich KMGrubb RL JrChicoine MRMoran CJCross DT III: Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 37:149014962006

    • Search Google Scholar
    • Export Citation
  • 3

    Handa JMatsuda INakasu SNakano Y: Agenesis of an internal carotid artery: angiographic, tomographic and computed tomographic correlation. Neuroradiology 19:2072111980

    • Search Google Scholar
    • Export Citation
  • 4

    Houkin KKamiyama HAbe HTakahashi AKuroda S: Surgical therapy for adult moyamoya disease. Can surgical revascularization prevent the recurrence of intracerebral hemorrhage?. Stroke 27:134213461996

    • Search Google Scholar
    • Export Citation
  • 5

    Karasawa JTouho HOhnishi HMiyamoto SKikuchi H: Long-term follow-up study after extracranial-intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease. J Neurosurg 77:84891992

    • Search Google Scholar
    • Export Citation
  • 6

    Kuriyama SKusaka YFujimura MWakai KTamakoshi AHashimoto S: Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey. Stroke 39:42472008

    • Search Google Scholar
    • Export Citation
  • 7

    Kuroda SHoukin K: Moyamoya disease: current concepts and future perspectives. Lancet Neurol 7:105610662008

  • 8

    Kuroda SHoukin KIshikawa TNakayama NIkeda JIshii N: Determinants of intellectual outcome after surgical revascularization in pediatric moyamoya disease: a multivariate analysis. Childs Nerv Syst 20:3023082004

    • Search Google Scholar
    • Export Citation
  • 9

    Lee JHOh CWLee SHHan DH: Aplasia of the internal carotid artery. Acta Neurochir (Wien) 145:1171252003

  • 10

    Quint DJSilbergleit RYoung WC: Absence of the carotid canals at skull base CT. Radiology 182:4774811992

  • 11

    Scott RMSmith ER: Moyamoya disease and moyamoya syndrome. N Engl J Med 360:122612372009

  • 12

    Suzuki JKodama N: Moyamoya disease—a review. Stroke 14:1041091983

  • 13

    Suzuki JTakaku A: Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:2882991969

    • Search Google Scholar
    • Export Citation
  • 14

    Yamauchi TTada MHoukin KTanaka TNakamura YKuroda S: Linkage of familial moyamoya disease (spontaneous occlusion of the circle of Willis) to chromosome 17q25. Stroke 31:9309352000

    • Search Google Scholar
    • Export Citation


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