KidsMD Health Topics

Moyamoya Disease in Children

  • When we hear that someone has had, or is at risk for, a stroke, we tend to immediately picture an older person. “After all,” we may think, “a stroke is something our parents and grandparents might face—not our children.” Unfortunately, pediatric stroke is a very real risk for many kids with certain cerebrovascular disorders, including Moyamoya disease.

    Moyamoya disease, which is also known as Moyamoya syndrome, is a rare but very serious condition in which the walls of the internal carotid arteries—the vessels that supply blood to important areas of the brain—become thickened and narrowed. This causes the flow of oxygen-rich blood to the child's brain to gradually slow down, and makes it more likely that a blood clot will form.

    This reduced blood and formation of blood clots are major risk factors for either a transient ischemic attack (TIA) also called a “mini-stroke,”or a full-fledged stroke.

    • “Moyamoya” means “puff of smoke” in Japanese. The disease gets its name from the wispy, tangled appearance of the new blood vessels that emerge in the brain (as the body attempts to compensate for the inadequate blood supply).
       
    • Moyamoya disease is a progressive condition, meaning that symptoms worsen over time and the child's chances of suffering a stroke increases.
       
    • The only proven treatment for is surgery to create a healthy, adequate new supply of blood for the impacted areas of the brain.
       
    • Boston Children's Hospital is one of the only centers in the world with expertise in treating Moyamoya disease. Our doctors have invented a highly effective surgical procedure called pial synangiosis to introduce a sufficient new source of blood vessel growth. 

    The detailed information on the following pages will help you gain a better understanding of Moyamoya disease, and will provide a clearer picture of what lies ahead.

    How Boston Children’s Hospital Boston approaches Moyamoya disease

    Boston Children’s has a long and distinguished history of caring for children with complex diseases and disorders of the brain, spine and central nervous system. Experts in our Department of Neurosurgery are regarded as an international leaders in understanding and treating pediatric Moyamoya disease, and we have a specialized program devoted entirely to this rare but life-threatening disorder.

    The only treatment for Moyamoya disease that’s proven to be effective in the long term is surgery. Boston Children's neurosurgeon R. Michael Scott, MD, has pioneered a surgical procedure called  pial synangiosis that directly affixes a healthy donor artery to the affected area of a patient’s brain. In 1985, Scott performed the first of these surgeries on a child with Moyamoya disease. Over the past 25 years, he and Edward Smith, MD, director of Pediatric Cerebrovascular Surgery at Children’s, have used the pial synangiosis procedure to treat more than 400 patients with Moyamoya.  

    Groundbreaking surgery for Moyamoya disease

    Watch a webcast of an actual pial synangiosis procedure here at Boston Children's.

    Moyamoya disease: Reviewed by Edward Smith, MD
    © Children’s Hospital Boston, 2011

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The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”
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