Vein of Galen malformations are a very rare type of condition that affects the blood vessels of the brain and it occurs during embryonic development. This condition results from an abnormal communication between the arteries and the veins in the developing brain. The Vein of Galen is a large vein that is located deep in the base of the brain. Normally blood will flow slowly through the capillaries to allow the exchange of oxygen into the surrounding tissues. With this type of malformation the capillaries are missing. This allows the blood to flow directly from the arteries to the veins. With this type of flow it does not slow down and will not allow the release of oxygen and nutrients. The rush of blood from the brain can cause many problems. Since the blood flows almost straight to the heart so rapidly, the heart can become overwhelmed, thus possibly leading to congestive heart failure. The faster flow of blood may also interfere with the normal blood drainage, which may lead to hydrocephalus. This malformation is rare and develops in utero. This malformation is caused by the blockage of the normal flow or absorption of cerebral spinal fluid. Vein of Galen malformations can be placed in three categories. Group one is neonates that develop congestive heart failure and a noise made by blood turbulence. This is called an intracranial bruit. The second group is infants that present with developmental delays and abnormal enlargement of the head. The third group is comprised of older children who will suffer from possible seizures or epileptic fits. They are in danger of possible strokes. They will also have an enlarged head and have difficulties learning.
It is possible to detect these kinds of abnormalities during antenatal ultrasound scans. Most often it does not appear until after birth when the child experiences rapid heart failure. In severe cases while in utero the baby will have difficulty coping with the abnormal shunt and this will cause the baby to die. If the child survives through birth they will rapidly go into heart failure that can be treated medically. Even with medical treatment in some cases the heart failure is so severe that other organs will be affected and the baby will not survive. Those children who do not develop heart failure will develop the hydrocephalus. This can cause the child to be floppy and sometimes have slight developmental delays. Treatment for these children is possible when they are a few months of age. And although their heads will remain large, over time as they grow their bodies will catch up with and become proportional to their head size.
Embolization is the most effective treatment for this type of malformation. A small catheter is fed through the arteries until the defect is located. Materials will be placed by the surgeon in the artery to block the blood flow to form a clot. The materials used range from fast acting glue to small particles or coils. Although this procedure is not 100% successful on the first attempt the subsequent efforts will gradually close off the remaining blood flow. After treatment is successful patients will often make rapid advances in their development and may go on to lead normal healthy lives. Those children who do not receive treatment will become at risk for developing permanent brain damage. The permanent high pressure within the vessels may also lead to the development of seizures or stroke.
It is possible to detect these kinds of abnormalities during antenatal ultrasound scans. Most often it does not appear until after birth when the child experiences rapid heart failure. In severe cases while in utero the baby will have difficulty coping with the abnormal shunt and this will cause the baby to die. If the child survives through birth they will rapidly go into heart failure that can be treated medically. Even with medical treatment in some cases the heart failure is so severe that other organs will be affected and the baby will not survive. Those children who do not develop heart failure will develop the hydrocephalus. This can cause the child to be floppy and sometimes have slight developmental delays. Treatment for these children is possible when they are a few months of age. And although their heads will remain large, over time as they grow their bodies will catch up with and become proportional to their head size.
Embolization is the most effective treatment for this type of malformation. A small catheter is fed through the arteries until the defect is located. Materials will be placed by the surgeon in the artery to block the blood flow to form a clot. The materials used range from fast acting glue to small particles or coils. Although this procedure is not 100% successful on the first attempt the subsequent efforts will gradually close off the remaining blood flow. After treatment is successful patients will often make rapid advances in their development and may go on to lead normal healthy lives. Those children who do not receive treatment will become at risk for developing permanent brain damage. The permanent high pressure within the vessels may also lead to the development of seizures or stroke.
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