What is the definition of Vein of Galen Aneurysm?
Vein of Galen aneurysm is a rare form of arteriovenous malformation in which the embryonic precursor to the vein of Galen, a vein at the base of the brain, dilates causing too much blood to rush to the heart. This can lead to rapid heart failure. Other features may include increased head circumference resulting from hydrocephalus, unusually prominent veins on the face and scalp, developmental delay, persistent headache, and other neurological findings.
Vein of Galen aneurysm is often recognized on an ultrasound late in pregnancy. In other cases, it is diagnosed after birth. Although the exact cause remains unknown, this condition appears to result from a defect in early fetal development. Treatment is aimed at decreasing the blood flow through the malformation while maximizing the blood supply to the brain. Minimally invasive surgical techniques are preferred, such as endovascular embolization.
What are the alternative names for Vein of Galen Aneurysm?
- Ectasia or varix of the vein of Galen
- Galenic arteriovenous malformation
- Vein of Galen aneurysm malformation
- Galen vein aneurysm
What are the causes for Vein of Galen Aneurysm?
Vein of Galen aneurysm originates during a baby's early prenatal development (usually during weeks 6-11). It involves a large vein at the base of the brain that is formed early in gestation and is normally replaced by the vein of Galen. In children with this condition, high-pressure, high-speed blood from the arteries flows directly into this precursor vein, preventing a true vein of Galen from forming.
Vein of Galen aneurysm has been associated with capillary malformation-arteriovenous malformation (CM-AVM) syndrome, a newly recognized autosomal dominant disorder caused by mutations in the RASA1 gene in a small number of families.