Moyamoya and Vascular Anomalies

Moyamoya disease is a very rare condition in which the walls of the internal carotid arteries - which supply blood to important areas of the brain - become thickened. This gradually slows the flow of blood to the brain and increases the likelihood of blood clot formation, both of which can lead to strokes and transient ischemic attacks.

In this condition, small blood vessels also form a network of "side roads" trying to supply oxygen to the oxygen-starved areas of the brain once served by the narrowed arteries. These many tiny blood vessels show up clearly on an angiogram, explaining the name for the disease; in Japanese, "moyamoya" means puff of smoke.

More fragile than normal blood vessels, arteries in this network can also break and bleed into the brain, causing hemorrhages (In the brain, hemorrhage can be even more dangerous than elsewhere in the body, as brain tissue becomes irritated and inflamed and the pressure inside the skull increases), in certain patients.

Moyamoya Treatment

Various surgical procedures are available and can be divided into two groups depending on whether they involve direct or indirect anastomosis between the blood vessels. In some patients, if micro aneurysms are identified, endovascular occlusion of the vessel can be performed, particularly in association with bypass surgery.

Indirect procedures are generally preferred for children under the age of 10 and bypass procedures are generally performed in older children and adults. In the latter groups, the two types of procedures (indirect and direct) may also be combined.

Without surgery, the majority of individuals with moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of arteries, and can be fatal as the result of intracerebral hemorrhage.

Surgical treatment of moyamoya disease using the various surgical techniques have been shown to be safe and effective in reducing ischemic events in children and potentially for reducing hemorrhagic events. Post-revascularization angiography and MRI studies often reveal a reduction in moyamoya vessels that closely parallels improvement in symptoms.

The pediatric neurosurgeons at the Florida Hospital for Children treat many patients with Moyamoya Syndrome and Moyamoya Disease and can provide expert care and treatment for your child.  To get started call us at 407-236-0006 and a skilled Children’s Care Coordinator will work with you and your physician’s office to coordinate your child’s care, answer your questions and explain the next steps. Their services are complimentary.

View Dr. Trumble's clinical presentation on Vascular Anomalies. (Viewer Discretion Advised: The presentation contains surgical images)