• Moyamoya Disease

    What is moyamoya disease? 

    Cerebral angiogram depicting the 'puff of smoke' pattern of Moyamoya diseaseMoyamoya disease is a special type of intracranial stenosis. It is characterized by the blocking of the main arteries to the brain (usually the carotid arteries) just as they enter the skull. The brain attempts to overcome this blockage and reduction in blood flow by enlarging other smaller blood vessels in a process called forming "collaterals." In patients with moyamoya disease, the enlargement of some of the vessels at the base of the brain leads to a pattern on an angiogram that somewhat resembles a "puff of smoke." Moyamoya disease is most common in the Asian population and the term moyamoya comes from the Japanese word for "puff-of-smoke."

    Patients with moyamoya disease are prone to strokes and brain hemorrhages. Patients may first require medical attention as children or adults. Symptoms may also vary widely. Medical treatments are often ineffective and surgery is frequently required. The goal of surgery is to provide more blood flow to the brain in order to decrease the future risk of stroke. Several procedures or combinations of procedures may be tried. The most common include:

    • Encephalo-duro-arterio-synangiosis (EDAS) involves placing an unaffected scalp artery directly on the brain and allowing connections (collaterals) to develop between the artery and the existing brain arteries, thus improving blood flow to the brain.
    • Intracranial to extracranial (EC-IC) bypass creates a direct connection between a scalp artery and a brain (intracranial) artery.
    • Encephalo-myo-synangiosis (EMS) uses muscle from the side of the head (temporalis muscle), which has a good blood supply, to accomplish the same purpose as EDAS.
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