Vein of Galen Malformations

  • One family’s story

    Through the nonprofit Partners in Health, 4-month-old Rolensky of Haiti came to Boston Children’s Hospital with heart failure caused by a large vein of Galen malformation (VOGM) deep in his brain. Two embolization procedures sealed off the malformed vessels, allowing Rolensky to make a dramatic recovery. Read more.

     

    Vein of Galen malformations (VOGMs) are a type of arteriovenous fistula (AVF), a rare blood vessel abnormality in which arteries connect directly with veins, bypassing the capillaries. VOGMs involve a large vein deep in the brain and are formed during early prenatal development.

    VOGMs are often noticed on a prenatal ultrasound late in pregnancy, and in other cases are diagnosed after birth. Symptoms may include:

    • heart failure, often within the first day or two of life
    • increased head circumference, resulting from hydrocephalus
    • unusually prominent veins on the face and scalp
    • failure to meet developmental milestones
    • persistent headache
    • in very rare cases, a bleed in the brain

    VOGMs can result in severe neurologic problems and may even be life-threatening if they are not diagnosed and treated early. When treated, however, the majority of children go on to live active and full lives.

    Why choose Boston Children’s Hospital?
    The Cerebrovascular Surgery and Interventions Center is one of the few pediatric centers in the world that specializes in treating VOGMs. Our safety record is unparalleled, and we use minimally invasive, catheter-based procedures in most cases. To ensure treatment success, we have pioneered the practice of performing an additional angiogram before waking patients from anesthesia.

    We bring together an unusually large number of specialties to care for your child, matched by few other hospitals. They include pediatric specialists in vascular anomalies, neonatal intensive care, cardiology, neurology, neuroanesthesiology, neurointerventional radiology and neurosurgery, as well as staff in our medical-surgical intensive care unit, whose expertise is critical in ensuring the best outcomes.

    Our physicians work closely and attend weekly conferences with the Vascular Anomalies Center at Boston Children’s. Through active research, we are constantly exploring methods to make a more precise diagnosis of VOGM and seeking new treatments aimed at safeguarding normal blood flow to the brain while minimizing flow through the VOGM itself.


  • Vein of Galen malformations (VOGMs) originate during a baby’s early prenatal development. They involve a large vein at the base of the brain that is formed early in gestation and is normally replaced by the true vein of Galen.  In children with a VOGM, high-pressure, high-speed blood from the arteries flows directly into this precursor vein, preventing a true vein of Galen from forming. The precursor vein enlarges dramatically due to the abnormally elevated flow and is visible on imaging studies.

    VOGMs sometimes, but not always, occur in conjunction with vascular anomalies affecting other parts of the body. They generally are not inherited.

    In VOGMs, as in other arteriovenous fistulae (AVFs), blood flows directly from arteries into the veins without traveling first through the capillaries. This causes a rush of high-pressure blood into the veins, setting off a series of problems:

    • In some newborns, the rush of blood toward the heart and lungs forces the heart to work overtime to get blood to the rest of the body, causing congestive heart failure.
    • Blood pressure in the arteries from the heart to the lungs may rise, causing a serious condition called pulmonary hypertension.
    • The abnormally high pressure in the brain’s veins can prevent the infant’s entire brain from being drained adequately. This can lead to widespread brain injury and sometimes severe loss of tissue throughout the brain.
    • Infants with VOGMs who do not develop symptoms in the first few days of life may, over the next few weeks or months, develop hydrocephalus, or enlargement of the head. This occurs when an enlarged VOGM blocks the normal flow or absorption of cerebrospinal fluid, increasing pressure in the venous sinuses (channels that collect venous blood). In such cases, the most visible symptom is an increased head circumference.

    Learn more by clicking the Test and Treatment tabs above.

  • Because vein of Galen malformations are usually diagnosed in young infants, we like to avoid the use of x-rays. For that reason, magnetic resonance imaging (MRI) is the main imaging technique we use to visualize the malformations, understand their structure and assess the status of the surrounding arteries and the overall condition of the brain. MRI is noninvasive and uses a strong magnetic field, radio waves and advanced computer processing to produce 2- and 3-dimensional images of the head, neck and brain. Read more about MRI.

     

    However, a variety of other techniques may be useful in particular cases. Specific tests may include:

    Cerebral angiography

    Cerebral angiography, sometimes called cerebral arteriography or catheter angiography, produces the most detailed images of the arteries and veins of the neck, head and brain, using live x-rays. We rarely perform angiography purely as a test, but instead use it to guide treatment of the malformation through embolization.

    • The neuroradiologist or neurointerventionalist inserts a small needle into the femoral artery in the groin and introduces a soft, thin wire that guides a catheter up to the arteries near the brain area to be imaged.
    • A special dye (known as contrast) is injected through the catheter, allowing the radiologist to more clearly see the malformation and the surrounding arteries and veins. The catheter also can be used to deliver treatments from within the vessels.
    • Cerebral angiography can take 30 minutes to several hours and is usually done under general anesthesia. After the angiogram, patients must lie flat for four hours in the recovery room, but can sleep, watch TV or use a handheld device, with parents close by.
    • We ask patients not to do vigorous exercise for several days after angiography, but they can return to school as soon as they feel ready. Read more about angiograms.

    CT Angiography (CTA)

    CT angiography (CTA) uses the technology of a conventional CT scan, along with an injected dye, to generate images of the blood vessels of the upper chest, neck and brain.

    • CTA generates images somewhat similar to those seen with cerebral angiography, but since the dye is injected into a vein through a standard IV, rather than into an artery, CTA is less invasive.
    • During this test, the child lies still on a table that slides slowly through a donut-shaped device. A computer constructs 3D images of the blood vessels from the CTA images.
    • Young children may need sedation to keep them still.

    Magnetic resonanc angiography (MRA)

    Magnetic resonance angiography (MRA) is a group of imaging techniques based on MRI technology, used to evaluate blood vessels in the brain, head and neck. Unlike CT angiography, many MRA scans do not rely on contrast injected into the veins to generate images of the vessels, although contrast injections are sometimes used. Like conventional MRI, MRA avoids the use of x-rays.

  • The goal of treating a vein of Galen malformation (VOGM) is to eliminate or decrease as much of the blood flow through the VOGM as possible, while maximizing the blood supply to the brain. In almost all cases, we use endovascular embolization, a minimally invasive, catheter-based procedure. Recovery is usually rapid, and most children can go on to live full and active lives. We have pioneered the practice of performing an additional angiogram before waking the child from anesthesia to verify that the treatment was successful.

    If your child is scheduled for endovascular embolization, you will receive specific information on how to prepare. Because general anesthesia is used in endovascular embolization, your child may need to stop eating, drinking and sometimes taking medicines for a certain period of time.

     

    Upon arrival, plan to spend about four hours meeting with nurses, the anesthesiologist and the neurointerventionalist. Children can bring a favorite toy or blanket into the procedure room, and parents can stay close by, in the family waiting area.

     

    • To begin the embolization procedure, an interventional neuroradiologist inserts a catheter (a thin, flexible tube) into an artery in the child’s groin through a tiny incision. Under x-ray guidance and with the help of soft wires, the catheter is advanced up the aorta (the main artery in the middle of the body) through the arteries leading to the VOGM. Multiple angiographic images may be taken to determine which vessels need embolization.
    • The neurointerventionalist then threads a smaller microcatheter through the first catheter to inject specialized materials into the vessels of the VOGM. These materials close off blood flow through the malformation from the inside and can vary from a fast-acting, glue-like compound to soft metal coils. We continually adapt new materials and devices designed for adults for use in children.
    • When the procedure is done, we remove the catheters, place a bandage over the site on the groin and transfer the child to the recovery room or to the ICU for observation.
    • To maximize safety, embolization is often performed in more than one procedure. Complications are rare, and our imaging protocols have been adjusted to minimize children’s exposure to ionizing radiation (x-rays).
    • No patient with heart failure is too young or too small to be treated: To date, our youngest patient to undergo embolization was a 35-week premature infant weighing 2 kg (4.4 lbs).
    • Most children have no pain or other symptoms with embolization, and most are able to leave the hospital within a few days. Some children will need to spend several days in the ICU for observation. Parents are welcome to stay with their children in the recovery room, the ICU and the hospital floor rooms afterward.
    • After discharge, we ask children to return for a follow-up office visit within a few weeks. Follow up may be in part through video teleconference for patients who live outside the Boston area.
  • Vein of Galen
    3D printed model of Vein of Galen.

    The Cerebrovascular Surgery and Interventions Center at Boston Children’s Hospital conducts ongoing research to better understand vein of Galen malformations. This work may pave the way for more precise diagnosis of VOGM and, in the future, more effective ways to close off the VOGM while preserving normal blood flow to the brain. We track our experience in a comprehensive patient database, allowing us to evaluate and compare techniques and improve our quality of care.

     

    3D printing to model brain and vascular anatomy

    Precision 3D models of VOGM—based on data from patients’ own brain scans—are allowing neurointerventionalists at Boston Children’s to plan and rehearse maneuvers in advance. The models are made by the Cerebrovascular Surgery and Interventions Center in collaboration with Peter Weinstock, MD, PhD, of the Simulator Program at Boston Children’s, using special digitally guided printers that lay down different types of molten plastic. Read more.

     

    Refining VOGM procedures

    A variety of novel techniques developed and trialed at Boston Children’s has allowed safe use of catheters and endovascular devices in infants’ tiny vessels. We also successfully adapted Onyx, an improved material used to treat cerebrovascular disease in adults, to VOGMs and other brain vascular malformations in children. We have an unparalleled safety record in treating children with this condition.

     

    Limiting radiation exposure

    New techniques have dramatically improved safety and reduced children’s radiation exposure during neurointerventions. Working closely with fluoroscopy equipment vendors to adjust and optimize technical factors, we have demonstrated that it is possible to achieve high-quality imaging at low radiation doses. Read more.

    See a list of our selected publications on cerebrovascular disease.

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