Vein of Galen malformation (VOGM) | Boston Children’s Hospital

A Vein of Galen malformation is an arteriovenous lesion, which means it’s in that category of conditions where there’s an abnormal direct communication between arteries, with high pressure blood, right into a vein. The vein that is accepting that high pressure blood is a vein that normally disappears during the course of fetal development, and that vein would have become the Vein of Galen, which is a normal deep vein in the brain, but in kids who have a Vein of Galen malformation, that fetal vein persists, it stays on, it’s often discovered while the mom is still pregnant, during the third trimester on an ultrasound, or it can be discovered very soon after birth with symptoms that can occur right after birth, or it can be discovered in the first few weeks and months of life with another set of symptoms that can occur there. Sometimes children who have this condition are not discovered even later than that, and within the first few months of life, the parents may notice that the veins of the face or the scalp are very prominent, and they may alert the pediatrician. Sometimes, the pediatrician may notice that the head circumference of the child is growing. All of these things are related to that high venous pressure. Vein of Galen malformations can cause different kinds of problems. One problem is that because you have very high pressure blood in the veins of the brain, the rest of the brain is trying to drain its blood normally back to the heart, but now it’s trying to compete with this high pressure arterial blood, and the result of that is elevated pressure throughout the veins of the brain, and if that is allowed to persist, that can result in major problems in brain development globally, and in some cases, you can actually start to get loss of the brain tissue, and very intense neurodevelopmental problems, so we do like to intervene fairly early on in treating them. In another set of children, they present soon after birth with heart trouble, and the reason for that is that the Vein of Galen malformation is such a high flow short circuit of the blood from the heart, going from the heart up to the malformation and right back to the heart, and not really supplying anything else on the way, it becomes a race with the heart pounding harder and harder and faster and faster, in the attempt to get enough blood to the rest of the brain. When the malformation has enough blood flow within it, in some newborns, they can present with heart failure within day or two of life, and so we prepare for that when we diagnose a Vein of Galen malformation during pregnancy, we make sure that the delivery is in a high-tech center that is able to care for children in a neonatal intensive care unit and watch for the symptoms of heart failure. When we see that, when there is that kind of cardiac trouble early in life, we actually have to do an intervention within a day or two of life, and do an embolization to try to close down as much of the flow into the Vein of Galen malformation as we can, really to control the heart failure, so we’re not so much trying to treat the brain in that intervention, as treat the heart failure. We sometimes need to bring the patients back a few times within the first few days of life, to really complete the shutdown of the flow as much as possible, to get the heart into a good place, and enable the patient to get out of the ICU, and develop normally for the first few months of life. We may then bring the patient back once they’re five or six months old, and can undergo a more complete procedure to really try to shut down the malformation entirely. This is a disease that really used to be a terrible diagnosis, until embolization techniques were developed to treat Vein of Galen malformation. In every case, we really look at where the child is developmentally, and what a safe time to intervene is, and try to prevent all of these later manifestations from happening.

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