Frequently Asked Questions FAQ/Information

A Vein of Galen Malformation (VOGM) is a type of cerebral arteriovenous malformation (AVM), which is a rare blood vessel abnormality inside the brain. In VOGMs, misshapen arteries in the brain connect directly with veins, instead of connecting with capillaries, which help slow blood flow. This causes a rush of high-pressure blood into the veins. The high pressure blood flow frequently leads to congestive heart failure and/or hydrocephalus. However, every VOGM is different. The severity of the condition and the severity of its correlating symptoms differ from child to child. Most children experience some stress on the heart or hydrocephalus before or shortly after birth, and most children have at least subtle symptoms by the time they reach one month old. Frequently, VOGM newborns are fed intravenously because eating could cause undue stress on the heart. VOGMs often require treatment in the form of medication or brain embolization prior to the child's first birthday.

VOGMs develop in the first trimester. Some genetic mutations have been linked to VOGMs, but a significant portion of VOGMs form without having a known genetic cause.

A VOGM is also called a Vein of Galen aneurysmal malformation (VGAM). VOGMs are sometimes called aneurysms. However, there is a slight difference. Aneurysms are enlarged arteries that are caused by weakening of the artery walls. VOGMs are enlarged blood vessels (not just arteries), and they become larger through the natural course of their development and not due to weakening of the blood vessel walls. 

It is very hard to predict the severity level of a VOGM prior to birth and if intervention will be needed right away. Most VOGM specialists encourage natural births for VOGM as well – the diagnosis itself is not a reason for C-section, and the decision is entirely up to the MFM and mom, in terms of what’s safest for mom and the baby from an obstetric perspective. Since the babies condition can decline in the first days of life, specialust encourage delivery at a high volume VOGM center incase intervention is needed right away. Often times if the baby is delivered local an inexperienced doctor must attempt treatment if the baby is not stable to be transferred. 

An interventional radiologist (IR) is the most common treatment provider for VOGMs. A neurosurgeon and neurologist, and frequently a cardiologist, will follow closely with your child's treatment for secondary conditions. 

Prior to surgery, imaging is required. The imaging is generally CT scans, MRIs with MRA/MRV, and angiograms. The vast majority of VOGMs are treated with endovascular surgery (surgery through the blood vessels) with embolization to close off the feeder veins into the Vein of Galen. The materials used to embolize the abnormal blood vessels are usually glues, coils, and onyx. VOGMs also often require medications to control correlated symptoms as well.

As a neonate, access through an umbilical arterial (UA) line is preferred to keep other access points viable for future surgeries. Three UA lines are commonly placed to increase the likelihood of successful access. With repeated use, arteries can collapse and make accessing the VOGM through the same artery impossible.

For infants and children, the femoral arteries or jugular vein are used.

According to a VOGM cohort study from 2006-2016 in the UK, with treatment there is a 33% chance of a good neurocognitive outcome, 33% chance of a poor neurocognitive outcome, and 33% chance of mortality. While nothing is guaranteed, finding the right specialist can significantly increase the likelihood of positive outcomes. In the resources section, there are linked articles with outcomes for a few other treatment providers.

Head circumference jump in percentile

Hydrocephalus

Congestive heart failure

Prominent facial veins

Vascular birthmarks  

A bruit (heartbeat) in the head

Some children never experience any complications outside of the VOGM; however, some children have had the following complications: 

Strokes

Brain bleeds

Brain damage

Seizures

Hydrocephalus

Developmental delays

How many children have you treated with VOGM?

What were your patients' outcomes?

When do you recommend treatment begin?

Would you be willing to consult with other specialists?

This question is specifically for American families.

Make sure your insurance has Out of Network benefits. If you have State Medicaid, please ask your questions in one of our Facebook groups. Medicaid can be tricky, but there are likely other families who have gone through it in your state. With that said, some states require an approval period prior to treatment. For example, in Alabama, the Out of Network doctor that you have chosen must have been approved for 90 days prior to receiving treatment for Alabama Medicaid to help pay for treatment.

After you have made sure that you have Out of Network benefits, ask a doctor who already sees your child to write a Letter of Medical Necessity and send it to your insurance company. The letter does not have to be from a specific doctor, but it should explain why treatment by the specific doctor you have chosen provides your child with the best opportunity for success. Like with Medicaid, you should ask your child's doctor to send the Letter of Medical Necessity to the insurance company well in advance of your treatment.

Fight every denial. Do not let the appeal periods on denials of benefits pass without attempting to fight the denials of coverage. The whole experience is horrible, but you have to keep fighting for your child and your family. The costs of treatment are exorbitant, and you cannot let an opportunity to lessen those costs pass you by.

Call your insurance and ask them if they have a Continuity of Care form that will cover a previously approved doctor due to the best interests of the child. As with the Letter of Medical Necessity, complete and submit a Continuity of Care Form as soon as possible.