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Vein of Galen

  • Overview

    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


  • In-Depth

    At Boston Children's Hospital, we know that a diagnosis of a brain abnormality, especially one as rare as vein of Galen malformation (VOGM), can be frightening experience. You probably have many questions and concerns, such as:

    • What are the risks and benefits of surgical procedures for VOGM?
    • Will my child have normal neurological and cognitive development?
    • What is the long-term outlook for my child?

    We’ve provided answers to commonly asked questions in the following pages, and our doctors will explain your child’s condition and treatment options to you in more detail during your appointment.

    What is a vein of Galen malformation (VOGM)?

    • VOGM is a form of an arteriovenous malformation (AVM), which is an abnormal connection between the arteries (blood vessels that carry blood from the heart to the body) and the veins (blood vessels that return blood to the heart).
    • All AVMs involve high-pressure, fast-flow blood that travels from the arteries directly into the veins (which are designed to handle low-pressure) and then directly to the heart at the same high-pressure and fast speed.  This rapid blood flow in AVMs is due to the absence of capillaries, small blood vessels located between the arteries and veins that act to slow down the flow of blood, allowing oxygen to enter the tissues.
    • VOGM affects the median vein, which is only present in the first 6 – 11 weeks of gestation before it disappears and contributes to the development of the true vein of Galen, one of the main deep veins of the brain. VOGMs are different than most other AVMs because they form so early in pregnancy and are usually diagnosed soon after the baby is born.
    • VOGM often presents itself with heart failure in newborns, although other organs can also be affected, leading to potential kidney and liver dysfunction.
    • Injury to the brain may or may not be present already at the time of diagnosis, even if the condition is discovered during pregnancy. The extent and severity of injury to the brain is an important factor to weigh in deciding how aggressively to treat VOGM. In order to avoid the development of such brain injury, we aim to treat VOGM by age 6 months whenever possible.
    • In a small number of cases, VOGM may be undiagnosed in infancy, and is thus left untreated.  Some of these undiagnosed children present later in childhood or even in early adulthood with new headaches or other neurologic symptoms.

    How serious is VOGM?

    VOGM is a life-threatening condition. However, survival rates have improved significantly since the 1980s, when more modern techniques used for treating the disorder (in particular, endovascular techniques) were developed.  Most children with VOGM will do very well if their brains have not been permanently injured and their other organs are intact, either developing entirely normally, or else having mild developmental delay.

    Unfortunately, if there is already multi-organ damage at the time of diagnosis, the chance that a newborn will recover is very low. If the brain has already been severly injured, there is a very low chance that the child will have normal neurological and cognitive development.

    Causes

    What caused my child to develop VOGM?

    VOGM is a congenital disorder, which means it is present at birth. This malformation develops early on in pregnancy because it forms in the median vein, which is only present during weeks 6-11 of fetal development. After that time window, the median vein disappears and contributes to the development of the true vein of Galen, one of the important deep veins of the brain.

    There is no single identifiable reason why a child develops this malformation. However, the majority of cases are sporadic, which means that the condition is not inherited and is not linked to any known problems or environmental exposures during pregnancy. In a minority of cases, there are mutations causing more widespread vascular malformations in the body, and some of these conditions are associated with an increased risk of VOGM.

    Symptoms

    What are the symptoms of VOGM?

    Symptoms of VOGM can show up at different ages and vary depending on the severity of the malformation.

    Neonates - newborns

    • The most severe forms of VOGM involve the highest and fastest flow of blood between arteries and veins.  
    • In these cases, the defect may is most often diagnosed in newbornswho experience rapid onset of heart failure, typically between days 1 and 3 of life. 
    • The increased stress on the heart may also lead to an increased workload on other organs, leading potentially to pulmonary hypertension, liver failure and kidney failure.
    • Significant brain damage may occur as a result of the brain’s inability to properly drain its venous outflow.

    Newborns - children

    Less severe symptoms of VOGM, which can show up at a variety of ages, include:

    • subacute to chronic cardiac problems that result from the change in circulation that occurs once the baby is born, when the heart struggles to pump enough blood to the entire body.
    • hydrocephalus (buildup of fluid in the brain) because the high venous pressures generated by the VOGM interferes with the normal flow and absorption of the cerebrospinal fluid. An early sign of hydrocephalus is increasing head circumference.
    • prominent veins on the face
    • As the high-pressure blood flows from the arteries directly to the veins in the VOGM, the entire system of veins in the brain becomes pressurized, the brain tissue starts to look for alternative ways to send blood back to the heart for re-oxygenation.
    • Normally, there are many very small connections between the veins draining the brain and  the head and neck and scalp veins. As the main venous pathways of the brain become pressurized by the VOGM, these connections with the face and scalp veins grow and the brain can start draining out of facial veins, making them appear prominent.
    • failure to meet developmental milestones:
    • A fast-flow VOGM that doesn’t cause heart failure might not be detected and treated early on. If this is the case, it is possible that the infant will stop meeting his expected developmental milestones because of the chronically high venous pressure interfering with normal brain perfusion.
    • These developmental delays can range from exteremly mild to potentially very severe limitations. They can include gross motor difficulties, fine motor difficulties, language delay, and general cognitive disabilities.

    Late childhood - early adulthood

    It is also possible that a VOGM will escape detection entirely until late childhood or early adulthood, appearing at that time with symptoms such as:

    Does VOGM affect life expectancy?

    Children who are treated early and have normal development or only mild delays lead full lives. There is no evidence to suggest that such patients have a shortened lifespan.  Most patients we treat fall into this category. Unfortuntely, newborns with severe multi-organ damage at the outset have a very low chance of recovery. If significant brain damage has already occurred at presentation, there is a very low chance of achieving meaningful neurocognitive development.

    Questions to ask your doctor

    You will probably have many questions on your mind before meeting with your child’s doctor. At the appointment, it can be easy to become overwhelmed with information and forget the questions you wanted to ask.

    Parents often find it helpful to jot down questions beforehand. That way, when you talk to your child’s clinician, you can be sure that all your concerns are addressed. Remember that physicians are open to learning from families too. Attend conferences, read up on updated materials and don’t be afraid to share what you have learned.

    Some questions you might ask include:

    • What treatment will my child need?
    • What are the risks of these procedures?
    • What’s in store for my child’s future cognitive development?
    • What other resources can you point me to for more information?
  • Tests

    At Children’s Hospital Boston, we know that an early and accurate diagnosis of vein of Galen malformation (VOGM) is crucial for the most effective treatment.

    How early can vein of Galen malformation (VOGM) be detected?

    Vein of Galen malformation (VOGMs) may be detected at a range of possible ages, from detection in utero on a prenatal ultrasound during the third trimester to detection soon after birth or even later.

    What diagnostic tests are used for VOGM?

    The diagnostic tests your child might require include:

    • Ultrasound: both during the third trimester of pregnancy and soon after birth (with dedicated head ultrasound), ultrasound is the critical first imaging test that leads to a diagnosis of VOGM. While a firm diagnosis of VOGM can usually be made using ultrasound, important details about the overall state of the brain and the morphology and complexity of the VOGM can only emerge from other kinds of imaging.
    • Magnetic resonance imaging (MRI): the main tool in diagnosing VOGMs, this imaging procedure uses a combination of large magnets, radiofrequency energy pulses, and sophisticated computer algorithms to produce detailed images of brain structure and give a detailed picture of the overall health of the brain.
    • Magnetic resonance venography (MRV): acquired at the same time as MRI, this is a tool that uses the same magnetic fields and radiofrequency energy to produce detailed images of the veins in the brain
    • Magnetic resource angiogram (MRA):  also acquired at the same time as MRI, this is another type of imaging test that focuses on producing detailed images of the arteries in the brain
    • Computerized tomography angiogram (CTA): an imaging tool that uses a combination of x-rays and powerful computer technology to create detailed images of the child blood vessels, with greater detail than can be achieved with MRA. However, unlike MRI/MRA/MRV, CTAs involve exposure to radiation from the x-rays, which we attempt to minimize, particularly in newborns. For this and other reasons, MRI/MRA/MRV are generally chosen over CTA scans.
    • Angiogram: cerebral angiograms play a critical role in the diagnosis and treatment of VOGM. Angiography is performed with a catetheter: a long, thin, flexible, hollow tube, which is usually inserted into an artery in the leg, and which is advanced, under imaging guidance, to the arteries that lead to the brain. In each artery, a special dye, contrast, is injected, providing detailed images of the blood vessels supplying the brain, as well as the blood vessels that make up the VOGM. Especially in young infants, angiography is almost always performed together with embolization: the procedure for closing down the VOGM.
    • Echocardiogram:  an ultrasound scan of the heart to assess its structure and function. Every child with VOGM will have this procedure to assess how much the heart function is being impacted by the malformation, if at all.
  • At Boston Children's Hospital, we know how difficult a diagnosis of vein of Galen malformation (VOGM) can be, both for your child and for your whole family. That's why our physicians take a compassionate, multidisciplinary team approach to treatment.

    From your first visit, you'll work with a team of professionals from intensive care, cardiology, neurology, radiology and neurosurgery who are committed to supporting all of your family's physical and psychosocial needs.

    When is treatment performed at Children's?

    If a newborn presents with heart failure, treatment can be life saving and must proceed without delay, even within the first few days of life, if necessary. If a newborn has VOGM but is not in heart failure, we try to wait for a few months before scheduling the first intervention to repair the malformation. Any embolization is much safer at age 5 or 6 months, and this age is still early enough to prevent negative impact on brain development.

    However, even at 5-6 months, it is often impossible to close a very large lesion in one session, so multiple sessions are usually required.  Subsequently, a child's treatment can extend, in staged fasion, until they are 2 or even 3 years old, depending on the complexity of the malformation. The goal at each stage is to allow them to develop normally.

    What are the treatment options?

    • Embolization: a minimally invasive procedure that has proven to be the most effective treatment option for VOGM.By feeding a narrow catheter through the arteries that lead to the defect, surgeons can introduce materials that block the blood flow through the malformation. The materials used can vary from a kind of fast-acting glue to small particles or coils.
    • Embolization is more difficult to perform in a newborn since the size of the catheters is dependent on the size of the blood vessel. A very small child requires very small catheters. Also, the amount of dye, or contrast, that can be injected in any one session, is limited by the weight of the patient, and only a small amount can be injected in newborns.
    • Embolization is usually done through the arteries. However, in some cases, the procedure will need to be done through the median vein itself. Treating VOGM trans-venously is usually riskier than treating through the arteries.

    It is often impossible to entirely shut down a VOGM in one procedure. However, by gradually closing off remaining areas of blood flow into the VOGM in subsequent stages, endovascular surgeons aim to eventually shut down the malformation entirely or nearly entirely. After successful embolization, it is our hope that patients can go on to full and active lives.

    What are the risks involved with surgery?

    The procedures, especially when done in the first few days of the baby's life, are very high-risk and carry a risk of death or disability.

    However, the prognosis for infants and children with VOGM has improved significantly over the years. This is largely due to improvements in treatment techniques. If VOGM is treated in cases where there is no damage to the brain and organ function is still intact, most children will do very well.

    What kind of follow-up care do children with VOGM need?

    The type of follow-up care depends on the child's individual circumstances.

    For example, if the child was diagnosed with VOGM as a newborn and received prompt treatment, the goal would be to treat the malformation thoroughly enough to help the child leave the Intensive Care Unit (ICU) and require only medical management.

    Similarly, if a child is born without heart failure but has a known VOGM, treatment would not begin until approximately age 5 or 6 months. During those 5 or 6 months, the child will be seen by his pediatrician weekly for head circumference measurements. The clinicians here at Children's will get a baseline MRI of the baby's brain, the baby will undergo a head ultrasound every few weeks, and the treatment team will continue to monitor his developmental milestones.

    This follow-up treatment can extend until the child is between the ages of 2 and 3 and even beyond, depending on the complexity of the malformation. The goal at each stage is to allow the child to develop normally, because a full and normal life is still possible even if with some continued flow through the malformation.  During the time the child is being treated, he or she will continue to have regular MRIs and neurologic exams, as well as ongoing assessments of general level of functioning.

    Once it appears that the VOGM has closed, or the treatment team decides that further treatment is not necessary because the flow through the malformation has been significantly reduced, and the risks of continued treatment outweight the benfits, he or she will continue to have regular visits with his pediatrician to assess ongoing development.

  • Research & Innovation

    At Children’s Hospital Boston, important technical breakthroughs are taking place in the approach to treating vein of Galen malformation (VOGM). 

    Although there are some VOG malformations that are impossible to treat due to severe brain or multiorgan injury at the outset, new developments are paving the way for even more effective treatment:

    • Glue-like substances that are injected into the brain are becoming less toxic and more controllable than before, while catheters are now even smaller and more flexible.
    • The development of new advanced variants of MRI/MRA/MRV has helped tremendously in assessing the overall condition of the brain, as well as the detailed morphology of the VOGM itself.
    • There is a significant amount of research in basic vascular biology about the underlying causes of VOG malformation, about broader vascular conditions of which VOGM may be one manifestation, and about how generally the blood vessels of the brain form. This may pave the way for earlier detection of VOGM as well as leading to supplemental medical treatment aimed at maximizing normal blood flow to the brain, while minimizing flow through the VOGM itself.

    Learn more about innovations taking place in Interventional Radiology at Children’s.

    Find out about clinical trials happening at Children's.

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