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Vein of Galen Malformations

A vascular malformation is an abnormal collection or tangle of blood vessels. The malformations restrict or alter blood flow and are associated with the degeneration of neurons. A particularly debilitating malformation is called a vein of Galen aneurysmal malformation (VGAM). VGAMs are rare, usually are found in very young patients (often in infants), and can have dramatic clinical consequences.

The vein of Galen, also called the great cerebral vein, is a large vein that drains oxygen-depleted blood from the brain. A VGAM occurs when blood flows from one or more arteries directly into the venous system of the brain because the normal intervening capillaries are absent. Capillaries are tiny vessels that slow down blood flow to allow for delivery of oxygen and nutrients to surrounding tissue. Their absence in VGAMs results in abnormally rapid blood flow into the Vein of Galen, which expands under the increased pressure.

Symptoms

The abnormally high level of blood flow through a VGAM often results in persistent and sometimes untreatable heart failure. Because of abnormal drainage of blood in the brain, VGAM may produce hydrocephalus ("water on the brain").

Diagnosis

Angiography, which provides an image of the blood flow in the brain, is the most important diagnostic tool for vascular malformations; it provides important information about both the location and structure of the vessels. Specialized forms of angiography also can be used to provide greater detail. Computed tomography (CT) and magnetic resonance imaging (MRI) scans often are used for diagnosis as well.

Treatment

Recently, prognosis of patients with VGAMs has improved, largely due to improvements in endovascular techniques. These techniques involve the use of a catheter, or tube that is inserted in an artery or vein and guided to the aneurysm at the site of the malformation. From there, surgeons have been able to stop up the arterial feeders with glue-like substances.

VGAMs are complex lesions, and should be addressed at major centers with experts experienced in their treatment.

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