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), 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 before 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 can also be called aneurysms. However, there is a slight difference. Aneurysms are enlarged arteries caused by a 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 the weakening of the blood vessel walls.
A neuro-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. 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, which treat 2-3 VOGM children a week. For the best possible outcome, please get a second opinion from one of the top specialists; their info can be found under resources.
Before surgery, imaging is required. The imaging is generally CT scans, MRIs with MRA/MRV, and angiograms. Most VOGMs are treated with endovascular surgery (surgery through the blood vessels) with embolization to close off the feeder veins into the Vein of Galen. Glues, coils, and onyx are used to embolize abnormal blood vessels. 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, making accessing the VOGM through the same artery impossible.
For infants and children, the femoral arteries or jugular veins are used.
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 veins 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, a 33% chance of a poor neurocognitive outcome, and a 33% chance of mortality. While nothing is guaranteed, finding the right specialist can significantly increase the likelihood of positive results. The resources section has 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, asking for a second opinion from a high-volume center like Boston Children’s Hospital or Mount Sinai in NYC is critical for the best chance at a good outcome being treated at a high volume center. 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 questions in one of our Facebook groups. Medicaid can be tricky, but some likely other families have gone through it in your state. With that said, some states require an approval period before treatment. For example, in Alabama, the Out of Network doctor you have chosen must have been approved for 90 days before receiving treatment for Alabama Medicaid to help pay for treatment.
After you have made sure you have Out of Network benefits, ask a doctor who 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. Treatment costs 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 covering a previously approved doctor due to the child's best interests. As with the Letter of Medical Necessity, complete and submit a Continuity of Care Form as soon as possible.
A build-up of fluid in the cavities deep within the brain. The extra fluid puts pressure on the brain and can cause brain damage. It's most common in infants and older adults.
Fontanelle: the soft spot on the top of a baby’s head, that can become hard or bulge if they have hydrocephalus.
EVD: External ventricular drainage (EVD) is a temporary method of draining cerebrospinal fluid (CSF) from the ventricles in the brain.
ETV: endoscopic third ventriculostomy ETV is a minimally invasive procedure that creates an opening in the floor of the third ventricle in the brain. This allows cerebrospinal fluid (CSF) trapped within the brain's ventricles to escape into its normal pathway.
CPC: choroid plexus cauterization procedure. is a procedure that reduces the choroid plexus (tissue that produces CSF) in two of the four ventricles inside the brain. This decreases the amount of fluid produced and may also reduce the strength of pulses that can cause the ventricles to enlarge. This makes the ETV procedure more likely to succeed in a child's brain.
VP Shunt: During the procedure, a shunt (a thin, flexible tube) is placed in the brain or spinal cord to drain the extra fluid. A one-way valve on the shunt regulates the flow of fluid. Many shunts can also be adjusted externally, using a magnetic device. Shunts need adjustment and replacement over time, and must be checked by a neurosurgeon on an ongoing basis.
Congested heart failure or High output heart failure: Heart failure occurs when the heart is not working to pump blood as well as it should, and gets backed up (congested) in the blood vessels. It is also called congestive heart failure. due to the malformation pumping too much blood to the heart it will become enlarged and go into heart failure.
Pulmonary hypertension: (PH) is abnormally high blood pressure in the arteries of the lungs. It is a serious condition that may lead to failure of the right heart ventricle. It is sometimes difficult to diagnose because many symptoms of PH aren’t exclusive to the condition but can be caused by a variety of health conditions.
Seizure: Seizures happen when brain cells fire or “talk” too much, temporarily disrupting the brain’s normal electrical signals. They’re quite common, especially in infants and young children, and they have a wide range of causes. Sometimes, seizures are triggered by a disease or injury, but for most children, there is no detectable cause. Sometimes other conditions, such as fainting or stroke, can look like seizures.
Stroke: Stroke is a general term that is used to describe an injury to the brain caused by either bleeding (referred to as hemorrhagic stroke) or a lack of oxygen due to vessel obstruction (referred to as ischemic stroke). A stroke usually implies some type of permanent injury to the brain. The term infarct or infarction may also refer to a stroke. When a stroke occurs in a newborn, it is called a neonatal stroke.
Brain bleed: Stroke is a general term that is used to describe an injury to the brain caused by either bleeding (referred to as hemorrhagic stroke) or a lack of oxygen due to vessel obstruction (referred to as ischemic stroke). A stroke usually implies some type of permanent injury to the brain. The term infarct or infarction may also refer to a stroke. When a stroke occurs in a newborn, it is called a neonatal stroke.
Join us on a heartwarming journey of compassion and determination as we lace up our sneakers and take steps towards a brighter future for children with the Vein of Galen malformation.