Vein of Galen Malformations (VOGM)

What Is a Vein of Galen Malformation?

The vein of Galen malformation (VOGM) is a rare blood-vessel malformation in the brain that develops before birth (usually during the first trimester) and is sometimes diagnosed in the womb or soon after birth.

The vein itself is a large deep vein at the base of the brain that takes away the oxygen-depleted blood. However, in this malformation, capillaries that normally slow blood flow and permit oxygen exchange with the surrounding tissues are missing, and so blood rushes directly from arteries into veins causing the heart to overwork.

If not diagnosed and treated early, VOGM can lead to a number of problems in infants, including: 

VOGMs belong to the family of disorders called arteriovenous malformations (AVM) and represent the most severe form of AVMs. The condition is sometimes referred to as a vein of Galen aneurysmal malformation (VGAM).

What Causes Vein of Galen Malformations?

The exact causes of VOGMs are not entirely known, a genetic mutation may play a part in this disorder. It is believed that VOGMs are not an inherited genetic trait.

What Are the Signs & Symptoms of Vein of Galen Malformations?

In the more severe cases, the blood overload goes almost directly to the heart and rapidly overwhelms it, leading to congestive heart failure. Depending on the severity of the condition, it may show up in several ways:

  • In newborns (typically within the first month) with congestive heart failure (sometimes the condition is noted in ultrasound before birth)
  • Infants with abnormally enlarged head and developmental delay (the enlarged head occurs because the malformation blocks the normal flow or absorption of cerebrospinal fluid)
  • Older children with a large head and possibly seizures and/or strokes; sometimes learning difficulties are noted

How Is a Vein of Galen Malformation Diagnosed?

A VOGM may be diagnosed in utero through an ultrasound. In most cases, however, a VOGM is diagnosed after birth via magnetic resonance imaging (MRI) and computed tomography (CT). Digital subtraction angiography, a test that uses x-rays and a special dye to see inside the arteries, is another diagnostic method a doctor can use.

How Is a Vein of Galen Malformation Treated?

Embolization is considered the most effective treatment for VOGMs. If a baby or child has been diagnosed with a VOGM, the treating neurointerventional physician will be a board-certified physician who has specific, sub-specialty training in treating high-level vascular abnormalities. A multi-disciplinary team consisting of specialists in Neurology, Cardiology, Neurosurgery and Critical Care is necessary to ensure that all aspects of care for VOGM patients are taken care of, including close follow up of the brain and heart function.

In embolization, the interventional neuroradiologist (neurointerventional surgeon) advances a small plastic tube (catheter) either through the umbilical artery (available during the first week after birth) or the leg artery at the groin, or directly from the carotid artery in certain cases. Catheters can also be inserted from the leg vein (at the level of the groin). The catheters are navigated to the location of the abnormal connection between the arteries and veins, and these abnormal connections are closed using injection of liquid “glue” and sometimes “coils” (miniature “slinkies” made of platinum).

2 pictures from an MRI showing a very enlarged, abnormal Vein (arrows) (Median prosencephalic vein, called Vein of Galen in early descriptions of the condition) due to the “short circuit” of arteries into the vein, causing the heart failure and buildup of fluid in the brain (asterisk).

MR angiogram of the head, viewed from the side, showing multiple arteries (arrowheads) directly connecting to the enlarged vein (red arrow).

Microcatheter advanced into one of the arteries directly connecting to the enlarged vein (red arrow). The tip of the microcatheter is seen as a small black dot (green arrowhead).  A liquid “glue” is injected through the microcatheter to close the abnormal connection of the artery to the vein.

X-ray of the head from the side.  The “glue” used to close the abnormal connections of the arteries and veins is seen as “black” areas (Green arrowheads).

Why Choose Lurie Children's?

Our sub-specialty trained pediatric neurointerventional radiologists specialize in treating infants and children of all ages and, collectively, have performed more than 2,000 minimally-invasive, image-guided procedures since the beginning of our program in 2005.

The neurointerventional team is led by Ali Shaibani, MD, a nationally renowned neurointerventional radiologist who has pioneered treatment of cerebrovascular diseases in children affecting the head, neck and spine using cutting-edge neurointerventional techniques. Learn more about our Neurointerventional  Radiology Program

Make an Appointment

To make an appointment, call 1.866.FETAL24 (1.866.338.2524).

The Chicago Institute for Fetal Health offers a team of multidisciplinary specialists for fetal and specific infant cases.