Moyamoya Disease

A rare progressive cerebrovascular disease in which the major arteries supplying the brain gradually narrow, leading to formation of fragile collateral vessel networks.

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Vascular and Endovascular Neurosurgery

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1Overview

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What is this Condition?

Moyamoya disease is a rare progressive cerebrovascular disorder characterized by gradual narrowing and occlusion of the major arteries supplying the brain (internal carotid arteries and their branches). The body compensates for this narrowing by developing fine, tortuous networks of collateral vessels; these networks appear as a 'haze' on angiography, which is how the disease acquired its name from the Japanese word meaning 'haze of smoke.' Unfortunately, these collateral vessels are inadequate and fragile, increasing hemorrhage risk. The disease is prominent in two age groups: in children, symptoms predominantly result from decreased cerebral blood flow (transient ischemic attacks, stroke, cognitive decline), while in adults, intracerebral hemorrhage is more common. Without treatment, recurrent strokes and hemorrhages may occur. Surgical revascularization (bypass) can significantly reduce stroke risk. Early diagnosis and regular monitoring are essential.

Symptoms

Transient ischemic attacks in children: sudden weakness in an arm/leg, transient speech or vision disturbance

Stroke: persistent weakness, speech disturbance, cognitive or perceptual difficulties

Attacks triggered in children by crying, rapid breathing, or blowing on hot food

Frequent headaches

Sudden severe headache, nausea-vomiting, altered consciousness in adults (signs of intracerebral hemorrhage)

Seizures and declining school performance, attention-learning difficulties

Diagnosis Methods

Initial evaluation includes contrast-enhanced brain MRI and MR angiography, which demonstrate narrowing of vessels supplying the brain, fine collateral vessel networks formed for compensation, and signs of previous ischemic events. The gold standard for definitive assessment is digital subtraction angiography (DSA), which provides the most detailed visualization of vessel status and disease staging. Additional tests such as SPECT/PET to measure cerebral perfusion in different brain regions and cervical-to-cerebral duplex ultrasound for follow-up blood flow assessment may be utilized.

Treatment Methods

The primary treatment for moyamoya disease is surgical revascularization to increase cerebral blood flow; medical therapy is aimed at symptom relief but does not arrest disease progression. Medications include antiplatelet agents (such as aspirin) and control of risk factors. Two main surgical approaches exist: direct bypass, where a branch of the carotid artery is anastomosed to a cerebral artery to provide immediate blood flow and is preferred in adults; and indirect bypass, where muscle or membrane tissues are placed on the brain surface to stimulate gradual formation of new vessels and is favored in children. In children, both methods are often used in combination. Surgical timing and technique are individualized for each patient; these procedures aim to increase cerebral blood flow and reduce stroke and hemorrhage risk.

Direct bypass (anastomosis of carotid artery branch to cerebral artery) — preferred in adults

Indirect bypass (gradual new vessel formation through muscle/membrane tissue) — more common in children

Combined application of both methods in children

Anticoagulant medication and control of risk factors (supportive care to alleviate symptoms)

Treatment Path

Related Treatments

The procedures and interventions most commonly explored with this condition.

4 links

Serebral bypass ameliyatı

Serebral bypass ameliyatı procedure

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Selective Cerebral Arteriography (4 Systems)

Separate catheter imaging of the four main brain vessels

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Selective Carotid Arteriography (Unilateral)

Selective Carotid Arteriography (Unilateral) procedure

Open treatment detail

Selective Vertebral Arteriography (Unilateral)

Selective Vertebral Arteriography (Unilateral) procedure

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References

Greenberg MS. Handbook of Neurosurgery. 10th ed. Thieme; 2023.
Yonekawa Y, Takagi Y, Khan N. Adult Moyamoya Disease. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 6th ed. Elsevier; 2011.
Khan N. Pediatric Moyamoya Disease. In: Spetzler RF, Kalani MYS, Nakaji P, Rangel-Castilla L, eds. Decision Making in Neurovascular Disease. New York: Thieme; 2018.
Spetzler RF, Kalani MYS, Nakaji P, eds. Neurovascular Surgery. 2nd ed. New York: Thieme; 2015.

This content is for informational purposes and based on academic sources; consult your physician for diagnosis and treatment.

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Moyamoya Disease

Vascular and Endovascular Neurosurgery

1Overview

What is this Condition?

Symptoms

Diagnosis Methods

Treatment Methods

Related Treatments

Serebral bypass ameliyatı

Selective Cerebral Arteriography (4 Systems)

Selective Carotid Arteriography (Unilateral)

Selective Vertebral Arteriography (Unilateral)

References

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Serebral bypass ameliyatı

Selective Cerebral Arteriography (4 Systems)

Selective Carotid Arteriography (Unilateral)

Selective Vertebral Arteriography (Unilateral)

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