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Moyamoya Disease

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PanVascular Medicine

Abstract

Moyamoya disease (MMD) is a rare idiopathic steno-occlusive disease of the internal carotid arteries resulting in collateralization from new fragile vessels at the base of the brain termed “moyamoya” vessels. It most commonly affects patients of Asian descent, but has been observed in all ethnic groups. The age of onset fits a bimodal distribution in the 5–9- and 40–44-year age groups. MMD most often presents with stroke-like symptoms in children and bleeding in adults. The treatment for MMD involves revascularization surgery to replenish blood flow to the diseased areas of the brain. Revascularization can be performed by “direct” bypass where an external vessel is anastomosed to a brain vessel to replenish flow immediately or by “indirect” means where a vascular donor tissue is surgically planted on or near the surface of the brain to encourage formation of new vasculature. Outcomes for moyamoya are favorable with revascularization surgery showing decreased rates of stroke reported in patients after successful revascularization. In this chapter we present the pathophysiology, epidemiology, classification, workup, treatment, and outcomes for MMD.

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Abbreviations

Cerebrovascular reactivity (CVR):

The ability of arterioles to contract or relax in response to a stimulus to alter cerebral blood flow as required.

Computed tomographic angiography (CTA):

A computed tomography method that visualizes arterial and venous vessels.

Computed tomographic perfusion (CTP):

Imaging method that utilizes an iodinated contrast agent to track the transit of blood through the cerebral vasculature.

Computed tomography (CT):

An x-ray imaging method that constructs a 3-D image of bones and soft tissue from a series of cross-sectional images made along an axis.

Diffusion weighted imaging (DWI) MRI:

An MRI method that can map the diffusion and disruption of molecules, mainly water, common in stroke.

Digital subtraction angiography (DSA):

A type of fluoroscopy technique used in interventional radiology to clearly visualize blood vessels in a bony or dense soft tissue environment.

Dual subtraction angiography (DSA):

A computer-assisted method that subtracts images of bone and soft tissue, revealing the cardiovascular system.

Encephalo-duro-arterio-myo-synangiosis (EDAMS):

A variation of EDAS where a wider craniotomy is performed to maximize exposure of the brain.

Encephalo-duro-arterio-synangiosis (EDAS):

An indirect method to improve collateral blood flow involving the transposition of a segment of a scalp artery onto the surface of the brain.

Fluid attenuation inversion recovery (FLAIR):

A pulse sequence used in MRI that negates the effects of fluids on the image, such as cerebral spinal fluid.

Mean transit time (MTT):

Time required for blood to pass through tissue.

MRI magnetic resonance imaging (MRI):

An imaging method that generates images of internal organs by measuring the atomic nuclei response of body tissues to high-frequency radio waves when placed in a magnetic field.

MRI perfusion (MRP):

Imaging method that uses gadolinium contrast agents or endogenous contrast generated by arterial spin labeling to track the transit of blood through the cerebral vasculature.

Oxygen extraction fraction (OEF):

The fraction of available oxygen extracted by the brain from the blood.

Positron emission tomography (PET):

A functional imaging technique that uses a radioactive substance to produce a 3-D image of functional processes throughout the body.

Single-photon emission CT (SPECT):

A nuclear medicine tomographic imaging technique that uses gamma ray emissions to track blood flow.

Xenon CT:

A diffusible radio-opaque gas that patients inhale, which enters brain tissue by diffusion. Xenon accumulation correlates to blood flow and can estimate CBF.

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Authors and Affiliations

  1. Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, USA

    Sunil V. Furtado

  2. Department of Neurosurgery, Stanford University School of Medicine, 300 Pasteur Drive, R281, Stanford, CA, USA

    Douglas J. Cook, Nitin Mukerji & Gary K. Steinberg

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  1. Douglas J. Cook
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  2. Nitin Mukerji
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  3. Sunil V. Furtado
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  4. Gary K. Steinberg
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Correspondence to Gary K. Steinberg .

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Editors and Affiliations

  1. Division of Cardiovascular Disease Department of Internal Medicine, Health Care Center Bitterfeld, Bitterfeld-Wolfen, Germany

    Peter Lanzer

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Cook, D.J., Mukerji, N., Furtado, S.V., Steinberg, G.K. (2015). Moyamoya Disease. In: Lanzer, P. (eds) PanVascular Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-37078-6_102

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