Stories about: cerebrovascular disease

Bad to the bone: New light on the brain’s venous system… and on craniosynostosis

cerebral veins and skull development in a normal child
Normal skull and brain venous development in a young child (courtesy Tischfield et al).

A recent study rocked the neuroscience world by demonstrating what in retrospect seems obvious: the brain has its own lymphatic system to help remove waste. A new study, from the laboratory of Elizabeth Engle, MD, at Boston Children’s Hospital, sheds light on another critical, little-studied part of the brain’s drainage system: the dural cerebral veins that remove and reabsorb excess cerebrospinal fluid.

The story of these vessels, the cover article in the next Developmental Cell, is a great example of lab scientists and physicians joining to make fundamental discoveries in biology. Strangely, critical clues come from children with craniosynostosis, a congenital malformation in which the skull plates fuse together too early in prenatal development, resulting in abnormal head shapes and, often, neurologic complications.

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Hold me, turn me: 3D printed models help doctors rehearse tricky cerebrovascular procedures

Vein of Galen-3D-20140418_Orbach-croppedFour children with life-threatening malformations of blood vessels in the brain appear to be the first to benefit from 3D printing of their anatomy before undergoing high-risk corrective procedures.

The children, ranging from 2 months to 16 years old, all posed particular treatment challenges: cerebrovascular disease often entails complex tangles of vessels in sensitive brain areas.

“These children had unique anatomy with deep vessels that were very tricky to operate on,” says Boston Children’s neurosurgeon Edward Smith, MD, senior author of the paper and co-director of the hospital’s Cerebrovascular Surgery and Interventions Center. “The 3D-printed models allowed us to rehearse the cases beforehand and reduce operative risk as much as we could. You can physically hold the 3D models, view them from different angles, practice the operation with real instruments and get tactile feedback.”

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A day in the life: A pediatric neurosurgeon’s vision

Ed Smith explains the moyamoya operation during a live webcast.

Lindsay Hoshaw contributed to this post.

It’s 7 a.m. and neurosurgeon
Ed Smith, MD
, is downing a Diet Coke as he reviews the MRIs of today’s patients. He sprints up a stairwell to greet his first patient in the pre-operating wing.

Thirteen-year-old Maribel Ramos, about to have brain surgery at Boston Children’s Hospital, sits in her bed fidgeting. Smith reassures her about the operation, promises they’ll shave off as little hair as possible, and gets Maribel to crack a smile by telling her he moonlights as a hairdresser.

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Curbing stroke’s inflammatory damage: A new target

Brain MRIs from mice after stroke. Mice lacking Hv1 (right panels) had a much smaller volume of infarcted tissue than normal mice. Hv1 can also be blocked chemically.

Whether it’s in adults or in children with clotting disorders or other conditions such as sickle-cell disease, a stroke can be likened to an atomic bomb. Just as ongoing radiation can do more damage than the bomb itself, the secondary damage of a stroke can devastate the brain.

In an ischemic stroke, accounting for nearly 90 percent of all stroke cases, it happens like this: When vessels supplying blood and oxygen to the brain are blocked by a narrowing or a clot, immune cells in the brain sense the low-oxygen conditions, suspect an invading organism and try to kill it by producing molecules known as reactive oxygen species or ROS’s. These, unfortunately, have an inflammatory effect that actually damages the brain further, injuring and killing neurons.

“Stroke produces inflammation, and that’s one of the main things people have been after in trying to reduce stroke damage,” says David Clapham, MD, PhD, chief of the Basic Cardiovascular Research Laboratories at Boston Children’s Hospital.

Right now there’s nothing that can do this. Most existing stroke drugs are aimed at preventing the stroke or dissolving blood clots once the stroke is happening – but they can’t deal with the aftermath.

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