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Pediatric Moyamoya Disease: Indirect Revascularization

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Pediatric Vascular Neurosurgery

Abstract

Moyamoya disease (MMD) is an idiopathic, chronic, occlusive cerebrovascular disease that involves bilateral stenosis or occlusion of the terminal internal carotid or proximal middle and anterior cerebral arteries. Development of basal collateral channels, including hypertrophy of the lenticulostriate and thalamoperforating arteries, results in characteristic “moyamoya vessels.” It is from the angiographic appearance of these vessels that the name moyamoya is derived, meaning “haziness” or “puff of smoke” in Japanese [1, 2]. Patients with moyamoya syndrome (MMS) present with identical clinical and angiographic features as those with MMD, but have an underlying associated condition, such as Down’s syndrome, neurofibromatosis, sickle cell disease, primordial dwarfism, or previous cranial irradiation [3].

Dr. Steinberg is a consultant for Qool Therapeutics, Peter Lazic US, Inc., and NeuroSave.

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Abbreviations

ACA:

Anterior cerebral artery

CBF:

Cerebral blood flow

CCA:

Common carotid artery

CVRC:

Cerebrovascular reserve capacity

DSA:

Digital subtraction angiography

DWI + ve:

Diffusion Weighted Image positive

ECA:

External carotid artery

EDAMS:

EncephaloDuroArterioMyoSynangiosis

EDAS:

EncephaloDuroArterioSynangiosis

EGPS:

EncephaloGaleoPeriosteoSynangiosis

EEG:

Electroencephalography

EMAS:

EncephaloMyoArterioSynangiosis

EMS:

EncephaloMyoSynangiosis

ICA:

Internal carotid artery

MCA:

Middle cerebral artery

MMA:

Middle meningeal artery

MMD:

Moyamoya disease

MMS:

Moyamoya syndrome

MBH:

Multiple burr holes

MR:

Magnetic resonance

OA:

Occipital artery

PCA:

Posterior cerebral artery

SPECT:

Single photon emission computed tomography

STA:

Superficial temporal artery

TIA:

Transient ischemia attack

Xe-CT:

Xenon-enhanced computed tomography

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Author information

Authors and Affiliations

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

    Mario K. Teo MD & Jeremiah N. Johnson MD

  2. Department of Neurosurgery, R281, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA, 94305-5327, USA

    Gary K. Steinberg MD, PhD

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  1. Mario K. Teo MD
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  2. Jeremiah N. Johnson MD
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Correspondence to Gary K. Steinberg MD, PhD .

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Editors and Affiliations

  1. Department of Neurosurgery, Methodist Neurological Institute, Houston, Texas, USA

    Abhishek Agrawal

  2. Department of Neurosurgery, Methodist Neurological Institute, Houston, Texas, USA

    Gavin Britz

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Teo, M.K., Johnson, J.N., Steinberg, G.K. (2016). Pediatric Moyamoya Disease: Indirect Revascularization. In: Agrawal, A., Britz, G. (eds) Pediatric Vascular Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-43636-4_16

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