WHAT IS A VOGM?
For a simplified explanation of a Vein of Galen malformation (VOGM) and its treatment, see these video From top VOGM Specialists.
one of the top VOGM specialists worldwide, Dr Orbach at Boston Children’s Hospital, explaining VOGM.
world renowned VOGM Specialist Dr Berenstein gives a TED talk on VOGM and how it is treated
Knowledge is power, parents and loved ones should educate themselves with their child’s condition in order to better advocate for their child.
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.
How Common is VOGM?
VOGM effects approximately 1 in 50,000 live births. there Are 3 million live births in the US a year, there for there are approximately 60 children born with VOGM a year in the US.
An neurointerventional 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. Due to the rarity and complexity of this condition, VOGM should not be treated by doctors who have only treated a few VOGM children in their carriers. It is critical to be treated by a high volume center like Boston Children’s Hospital or Mount Sinai in NYC who treat 2-3 VOGM children a week. For the best possible outcome please be sure to get a second opinion from one of the top specialist, their info can be found under resources.
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
Congestive heart failure
Prominent facial veins
A bruit (heartbeat) in the head
Some children never experience any complications outside of the VOGM; however, some children have had the following complications:
How many children have you treated with VOGM?
What were your patients' outcomes?
When do you recommend treatment begin?
most importantly, ask for a second opinion from a high volume center like Boston Children’s Hospital or Mount Sinai in NYC, for the best chance at a good outcome being treated at a high volume center is critical. contact info for Boston and Mount Sinai is under our Resource page.
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.
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