Vein of Galen
With the dramatic improvement in treatment options, caring for those with vein of Galen malformation has become a great story of hope. We can now offer many of these children completely normal lives and it gives me a huge amount of gratification to treat them because of that stark contrast between what was then and what is now.
Darren B. Orbach, MD, PhD, Neurointerventional Radiologist at Boston Children's Hospital
Vein of Galen malformations (VOGM) occur when veins and arteries near the center of a child’s brain form a direct connection with each other during early prenatal development, resulting in a vascular lesion called an arteriovenous malformation. The result is that high-pressure arterial blood flows directly into what should be the low-pressure venous system. The direct flow of high-pressure blood from the brain back into the child’s heart can lead either to congestive heart failure soon after birth or to significant neurological problems within the first year of life.
Here is some additional information about VOGM:
- VOGM is a congenital (present at birth) condition in which there is a vascular “short circuit” near the center of the brain, causing a rapid, uncontrolled flow of blood from the arteries to the veins, increasing the heart’s workload.
- VOGM is extremely rare, affecting between 1 in 100,000 to 1 in 1 million newborns. The disorder affects both genders equally.
- In its earliest form, VOGM usually presents itself as heart failure, although it can cause kidney and liver failure, too. If not treated within the first six months of life, potentially serious brain damage can result.
- VOGMs is a vascular malformation thought to develop between weeks 6 and 11 of the mother’s pregnancy. Although it can be detected on an ultrasound during the third trimester, VOGM is often not diagnosed until after the baby is born.
- No single, identifiable explanation about why babies develop VOGM has been established. The majority of cases are sporadic, which means there is no increased risk for siblings or relatives. However, recently, several conditions associated with vascular anomalies in different parts of the body have been shown to be associated as well with increased risk of VOGM.
- The best treatment option in most cases is embolization (a catheter-based treatment, in which a glue-like substance is injected into a blood vessel in the brain in order to block flow through the abnormal arteriovenous pathways).
- VOGM can be life-threatening. However, due to advances in our understanding of this condition and improvements in treatment options,the majority of children who undergo treatment for VOGM go on to live active and full lives.
How Children’s Hospital Boston approaches vein of Galen malformation
Children's is one of the few pediatric hospitals that specialize in the treatment of vein of Galen malformation. Our multidisciplinary approach allows us, unlike virtually every other hospital, to bring together world-renowned pediatric specialists in neonatal intensive care, cardiology, neurology, neurointerventional radiology, diagnostic neuroradiology, and neurosurgery.
When your child is treated here for VOGM, our expert clinicians will work closely together to monitor his or her development and assess progress at every stage. And we’ll be sure to involve you and your family throughout the treatment process.
Vein of Galen malformation: Reviewed by Darren B. Orbach, MD, PhD
© Children’s Hospital Boston; posted in 2012