Vein of Galen Malformation Defined

Vein of Galen Malformation Defined

Vein of Galen Malformation, often referred to as a vein of Galen aneurysm, is a rare blood vessel malformation in the brain that occurs before birth (affecting approximately one in one  million).

In this congenital vascular malformation, there are abnormal connections between the arteries (carries blood to the body) and deep draining veins (returns blood to the heart) specifically the vein of Galen, located in the base of the brain. With these types of malformations, the patient lacks capillaries to slow the blood flow  between arteries and veins. As illustrated below, without capillaries, blood is shunted directly in to the vein at a high output. This increases the workload of the heart. If left untreated, VOGM often results in abnormal brain development, mental retardation, strokes, seizures, paralysis, learning difficulties, hydrocephalus and ultimately cardiac failure and death.

Graphic courtesy of Center for Endovascular Surgery http://neuro.wehealny.org/endo/cond_vein-of-galen.asp

SONOGRAM

Diagnosis of VOGM aneurysm can be detected in-utero by the third trimester using a routine ultrasound examination. However, it’s important to note that many VOGMs go undetected until the baby is born.

SYMPTOMS

Symptoms of VOGM are often subtle and hard to detect, especially if the general practitioner, pediatrician or even pediatric cardiologist have never heard of this rare condition. Symptoms include high cardiac output, cardiac failure, hydrocephalus, seizures, headaches, prominent veins of the face, dark circles under the eyes, failure to thrive and in older children, developmental delay, nosebleeds and proptosis (bulging eyes).

TREATMENT

Standard surgical procedures have proven difficult in treating VOGM. Alternate treatments have been developed including endovascular embolization, which uses special coils or glue to carefully close off the arteries leading to the vein. During the procedure, a tiny incision is made in the groin and a catheter is inserted in to the femoral artery. Using fluoroscopic guidance, the surgeon moves through the circulatory system to the problem site and then deposits the coils, glue or balloon material to close off the artery.

This highly specialized procedure was pioneered by neuroradioligist, Dr. Alejandro Berenstein at the Center for Endovascular Surgery in St. Luke’s Roosevelt Hospital in New York. Only a few arteries can be closed off at one time so several procedures may be needed to reduce the vein of Galen down to a normal healthy size.

 

Before embolization (left); immediately after (middle) and 12 months following (right).Graphic via Center for Endovascular Surgery http://neuro.wehealny.org/endo/cond_vein-of-galen.asp 

 

A second treatment option involves a similar shunting of the arteries but instead of using the femoral artery as a gateway, a surgeon introduces a needle into the back of the head, localizing the venous system of the brain and then places trans-torcular coils in to the vein of Galen to occlude it. This procedure was developed at the University of Florida.

Insertion of needle into the venous system for placement of trans-torcular coil. Graphic via University of Florida.
http://www.neurosurgery.ufl.edu/patients/vein-of-galen-malformation.shtml

 

The treatment of Vein of Galen Malformation is highly specialized and should only be attempted by hospitals whose neurology team includes a neuorosurgeon and interventional neuroradiologist who have knowledge of and experience with VOGM.

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