PEDIATRIC NEUROSURGERY

Cerebrovascular disorders (Moya Moya)

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A Cerebrovascular disorder is an inherited disease in which certain arteries in the brain are constricted. Blood flow is blocked by the constriction, and also by blood clots (thrombosis).The blood vessels develop collateral circulation around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone to hemorrhage, aneurysm and thrombosis. On X-rays, these collateral vessels have the appearance of a "puff of smoke" ("moyamoya" in Japanese).

The disease causes constrictions primarily in the internal carotid artery, which travels from the neck up to the Circle of Willis inside the skull just under the brain. At the Circle of Willis, the internal carotid artery flows into the middle cerebral artery and the anterior cerebral artery. Moyamoya disease often extends to the middle and anterior cerebral arteries.

When the internal carotid artery becomes completely blocked, the fine collateral circulation that it supplies is obliterated. Patients often survive on the collateral circulation from the back (posterior) brain circulation from the basilar artery.

Drugs such as aspirin are usually given to prevent clots, but surgery is usually recommended. Since moyamoya tends to affect only the internal carotid artery and nearby sections of the adjacent anterior and middle cerebral arteries, surgeons can direct other arteries, such as the external carotid artery or the superficial temporal artery to replace its circulation. The arteries are either sewn directly into the brain circulation, or placed on the surface of the brain to re-establish new circulation after a few weeks.

Please contact NSPC (631) 983-8400 for more information or to schedule an appointment.