Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
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Monroe Carell Jr.
Children's Hospital
at Vanderbilt
2200 Children's Way
Nashville, TN 37232

(615) 936-1000

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What is Moyamoya?

Moyamoya is a disorder of the arteries of the brain. Typically, the internal carotid arteries progressively narrow and brain cells receive less blood and oxygen. Multiple small branching vessels form that have the appearance of a "puff of smoke" (moyamoya in Japanese). These new vessels are fragile and do not provide adequate blood flow. Patients with this condition can have strokes, seizures, and loss of neurologic function as a result. More than half of the cases of moyamoya have no known cause. The remainder can be due to conditions such as sickle cell disease, Down syndrome, and neurofibromatosis type I. Females are more than twice as likely as males to have moyamoya.


How is Moyamoya diagnosed?

Once a child exhibits symptoms suspicious for moyamoya, imaging is usually obtained to look at the brain blood vessels. MRI and magnetic resonance angiography (MRA) are often used as the initial way to look at the brain and blood vessels. Cerebral angiography is also used if the initial noninvasive studies suggest moyamoya. This test is the most accurate in demonstrating the brain blood vessels and establishing the correct diagnosis.

How is Moyamoya treated?

In general, children with moyamoya need more blood flow to the brain. Medical treatment often includes aspirin, which thins the blood slightly and promotes blood flow. Surgery may also help to increase brain blood flow. A variety of surgical techniques, including direct and indirect bypass, may help. A thorough consultation and medical evaluation are essential to properly diagnose moyamoya and establish a comprehensive treatment plan. Speech, occupational, and physical therapy also play key roles in most cases.

At Monroe Carell Jr. Children's Hospital at Vanderbilt, we are experts at diagnosing and treating children with moyamoya. Through our multidisciplinary center, we provide resources for comprehensive treatment, long-term follow-up and support for patients and families.

For more information, please contact us.

Last Edited: July 1, 2016
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