Stories about: moyamoya

Twin surgeries bring this family a stroke of luck

Ryan Earle is outgoing and upbeat, goofing around in his neurosurgeon’s office. He’s had two strokes, but he’s recovering steadily, a testament to the resilience of children’s brains. His twin brother Tyler, with him at the visit, has avoided a likely stroke altogether.

Both Ryan and Tyler, 8, have a rare brain disorder called moyamoya disease, in which the internal carotid arteries become narrowed, slowing the flow of blood to their brains. Without surgery, moyamoya poses a five-year stroke risk of 60 to 90 percent.

The boys’ moyamoya symptoms were subtle: “They would have a mild headache, then they’d throw up, but then an hour later they’d go to school and they’d be fine,” says their mother, Kathy.

Their pediatrician in Winnepeg, Canada, therefore, was not initially concerned.

But then Ryan, who used to make perfectly formed letters, suddenly couldn’t write. “I thought, ‘this is strange, maybe his hand is broken,’” Kathy recalls.

He also had weakness on one side of his body and was having trouble with language. “He couldn’t find the words to say ‘truck.’ He’d say, ‘those things,’” says Kathy. “The neurologist said he’d had a stroke.”

Ryan underwent an operation for moyamoya in a hospital in Winnipeg to try to restore blood flow to the left side of his brain. But then came a second stroke, worse than the first. It caused Ryan to lose part of his vision, made speech more difficult and partially paralyzed him, forcing him to use a wheelchair.

Ryan needed a second operation, on the right side of his brain. But the Winnipeg hospital was hesitant to move too quickly, wanting to give him time to recover from his stroke.

In the meantime, Tyler was also diagnosed with moyamoya, and imaging tests showed his disease was advancing. Like Ryan, both sides of his brain showed the classic “puff of smoke” (moyamoya in Japanese)—wispy, tangled blood vessels grown by a brain desperate for blood. He would need neurosurgery too.

The Earles had to act. “My perception was, it was a ticking time bomb waiting to go off,” says David, the boys’ dad. “Time was not on our side.”

Their research led them to confer with neurosurgeon Edward Smith, MD, of Boston Children’s Hospital’s Cerebrovascular Surgery and Interventions Center. He reviewed the boys’ scans and told the Earles that his team could operate on both sides of Tyler’s brain on the same day, rather than space out the operations, and that Ryan would not need to wait. “They had done it so many times, they were completely confident,” says Kathy. “They said ‘we can definitely go in.’”

Growing new blood vessels

twns with moyamoyaSmith planned to use an approach developed by his mentor, Michael Scott, MD, called pial synangiosis. First performed at Boston Children’s in 1985, it takes a healthy artery from the scalp and attaches it to the part of the brain that isn’t getting enough blood. The artery naturally takes root, providing a permanent new source of blood.

In May 2013, Ryan and Tyler had their back-to-back moyamoya operations—one on the right side of Ryan’s brain and one on each side of Tyler’s.

“A major consideration was that both sides could be done at the same time,” says David. “Dr. Smith operated in the morning, Dr. Scott in the afternoon.”

Fast-forward one year.

Today, at their one-year checkup, Smith shares the results of the boys’ follow-up brain angiograms, performed by interventional neuroradiologist Darren Orbach, MD, PhD. Both sets of scans show healthy vessels and blood flow, and both Tyler and Ryan have an excellent prognosis.

“You guys are so good at growing blood vessels,” says Kathy, grinning at her boys.

Ryan is clearly bouncing back from his strokes. He still needs a one-to-one worker at school, but his speech is better, and he no longer needs a wheelchair. His headaches are minimal. He’s fighting to catch up with Tyler—who recently won a provincial chess championship in Canada. Like his brother, Ryan is in a French immersion class at school.

“He’s very aware that Tyler can do some things he can’t,” says Kathy. “But he’s constantly improving.”

The Cerebrovascular Surgery and Interventions Center at Boston Children’s Hospital provides multifaceted care for children and young adults with moyamoya and other cerebrovascular diseases. To request a consultation, contact the Center at 617-919-1379 or email CVDSurgery@childrens.harvard.edu.

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Pediatric stroke: developing a better understanding through research

chh_2780_300x250Today at 10 A.M. Children’s Hospital Boston will broadcast online a live neurosurgery operation where doctors will treat a young patient with a rare neurological condition called moyamoya disease. In addition to providing the virtual audience with an up close view of surgeons performing a cutting edge treatment developed at Children’s, a panel of experts will comment on the procedure offering insight to the treatment, the disease itself and other related topics.

One of the panel’s speakers will be Michael Rivkin, MD, director of the Cerebrovascular Disorders and Stroke program at Children’s Hospital Boston, who will discuss cerebrovascular diseases and stroke treatment at Children’s. While pediatric stroke is a very serious risk for patients with moymoya, these patients are not the only children who can experience a stroke. Many children are at risk and research on the causes and treatment of pediatric stroke is still a developing field in pediatrics.

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Watch Children's neurosurgeons perform surgery online

treating moymoyA disease like moyamoya is so rare it only affects a few hundred children in the world. So few moyamoya cases are reported each year that many hospitals don’t have much of a track record or experience in treating the disease, in which the thickening of cerebral arteries reduces blood flow to the brain and require the formation of a network of tiny but weak arties to provide an ‘alternate route’ for the blood to reach the brain. These arteries are so small and closely aligned they resemble wisps of smoke on some tests. (Moyamoya means ‘puff of smoke’ in Japanese, hence the disease’s name.)

Children’s Hospital Boston, however, has been successfully treating kids with moyamoya for two decades using a special procedure developed by Children’s researchers and scientists.

As part of a webcast that will air live at 10 a.m. tomorrow, neurosurgeons from Children’s will telecast an operation on a young moyamoya patient, giving the virtual audience a bedside view of the surgical team’s cutting-edge procedure.

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