Moyamoya disease is a progressive cerebrovascular condition that gets worse over time, with narrowing and blockage of arteries (internal carotid arteries) that supply blood to the brain. The brain tries to make up for reduced blood flow by making new abnormal blood vessels, "collaterals", or moyamoya vessels. “Moyamoya” means “puff of smoke” in Japanese and refers to the wispy appearance of these abnormal blood vessels on imaging studies. While these blood vessels can try to help increase blood supply, they do not work well. People with moyamoya are at risk for having strokes.
Because moyamoya is a progressive problem, patients need treatment to reduce their risk of stroke. Without surgery, most people with Moyamoya disease will experience neurologic decline and strokes because the progressive narrowing of arteries does not allow enough blood and oxygen to get to the brain.
surgery for moyamoya
Pediatric Neurosurgery has a great track record of treating moyamoya in children.
We recently reported our results from over 100 surgeries in the Journal of Neurosurgery: Pediatrics. We describe how we perform the surgeries, and report on how children benefited from surgical treatment.
There are several types of surgery that can restore blood flow (revascularization) to the brain by bypassing blocked arteries and helping the brain develop new vessels to bring blood to the brain. Children usually respond well to revascularization surgery. The majority of children with moyamoya have no further strokes or related problems after surgery.
We typically perform indirect bypass with the superficial temporal artery and use the blood-vessel-rich outer layer of the lining of the brain, the dura, to help bring new blood supply to the brain affected by moyamoya. We have shown that this strategy is safe and effective in treating children with moyamoya.
The disease primarily affects children, but it can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as “mini-strokes”), frequently accompanied by weakness or paralysis affecting one side of the body, difficulty with speech, or seizures.
Children with moyamoya may also have other disorders, such as neurofibromatosis, sickle cell disease, or Down syndrome. With proactive screening, we aim to diagnose moyamoya on imaging studies before patients have strokes so that they can receive treatment to protect them from strokes. For children who are found to have moyamoya in the absence of these above disorders, we have our vascular genetics team screen for genetic mutations that may run in the family, so that patients, parents, and siblings are armed with information.