Vein of Galen Malformation

Vein of Galen Malformation

This malformation can sometimes be diagnosed with prenatal ultrasound but most commonly is detected when a newborn infant develops rapid heart failure. Other symptoms are hydrocephalus, developmental delay or seizure. The malformation is caused by a blood vessel in the brain that is present during embryonic development but supposed to disappear before the baby’s birth. Because the vessel persists in the brain it allows for a direct connection between the arterial and venous systems of the brain. So, rather than flowing through capillaries as it is supposed to the blood flows directly between the two systems. Because arteries flow faster than veins are capable of draining, this connection puts a great strain on the organs of the body, most notably the heart. That is another reason for the hydrocephalus – the brain cannot drain the blood fast enough and it builds up in the brain. The malformation needs to be treated to prevent continued symptoms and death. The old method (not very effective) of treating the problem was surgery, but today embolization is more commonly used and has a higher success rate. The purpose of embolization is to reduce the blood flow that is feeding the malformation. The malformation is gradually blocked off using either coils or glue which is introduced through a femoral catheter. Sometimes it takes more than one embolization procedure to treat the malformation. Prognosis is usually good if the disorder is caught and treated before developmental delays develop. I was surprised to find a support group on the web!

Reference:

Vein of Galen Malformation Support Group. Accessed October 27, 2009 at http://www.veinofgalen.org.nz/about_vein_of_galen/signs,_symptoms_and_diagnosis

Image:

This image is a sagittal MIP from a CT cerebral angiogram on an infant and shows a Vein of Galen malformation. The posterior superior sinus, transverse and sigmoid sinuses are enlarged.

Image from: Radiopaedia.org, Accessed on October 27, 2009 at http://radiopaedia.org/cases/vein-of-galen-tof-mrv