Abstract
Moyamoya disease (MMD) is a chronic progressive vascular occlusive disease causing bilateral stenosis of the distal internal carotid arteries and anterior and middle cerebral arteries. There is also an abnormal vascular formation of fragile collateral vessels at the base of the brain. See Fig. 103.1. Aneurysms can form in these collateral vessels [1, 2]. Normal cerebral blood vessels may vasodilate in response to chronic ischemia predisposing these patients to ischemia with decreased cerebral perfusion pressure. The term moyamoya derives from Japanese, meaning “haze-like,” a term that describes the puff of smoke angiographic appearance of the cerebral arterial collateral circulation. MMD is most prevalent in the East Asian population, particular in Japan, suggesting a possible genetic etiology. Ten to twelve percentage of patients have a familial predisposition with an autosomal-dominant inheritance [3]. However, MMD is seen less frequently in these populations after relocation to North America and Europe [4]. Patients who demonstrate the classical angiographic appearance of moyamoya, without any other risk factors, are known to have the disease, while those with any of the associated conditions are said to have the syndrome [5].
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Attali, A., Asif, A., Cook, S. (2018). Moyamoya Disease. In: Mankowitz, S. (eds) Consults in Obstetric Anesthesiology. Springer, Cham. https://doi.org/10.1007/978-3-319-59680-8_103
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DOI: https://doi.org/10.1007/978-3-319-59680-8_103
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