Vein of Galen Malformation

Vein of Galen Malformation

Article
Brain & Nerve
Heart & Vascular Health
+2
Contributed byMaulik P. Purohit MD MPHApr 11, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • VGM (Vein of Galen Malformation
  • VOGM (Vein of Galen Malformation)

What is Vein of Galen Malformation? (Definition/Background Information)

  • Vein of Galen Malformation (VGM or VOGM) is a rare disorder. It is a subtype of vascular malformations of the brain. In this condition, abnormal connections form between the arteries and the deep draining veins of the brain
  • The vein of Galen is a large vein that carries deoxygenated blood to the heart from the brain. The supporting fibrous wall to the vein is absent in VOGM, making the vein vulnerable to blood pressure changes
  • The signs and symptoms of this congenital malformation is manifested before or shortly after birth. The risk factors for developing Vein of Galen Malformation are unknown
  • However, this vascular malformation is characterized by the formation of tangled blood vessels in the brain. It is believed that certain genetic changes occur in the developing fetus during the first trimester, resulting in the abnormality
  • The symptoms of Vein of Galen Malformation are congestive heart failure in newborns, hydrocephalus, bleeding in the brain, seizures, and developmental delays. The potential complications of VOGM can include stroke and permanent disability
  • The condition can be diagnosed prenatally. After birth, a physical examination, an assessment of symptoms and imaging of the brain can aid in the diagnosis of Vein of Galen Malformation
  • Since Vein of Galen Malformation is present at birth, the affected newborn may need cardiac medications, oxygen therapy, or a ventilator till such a time that he/she can withstand surgery. A surgery may be performed to decrease the flow of blood through the vein of Galen, to reduce the strain on the heart
  • The prognosis of Vein of Galen Malformation is good with adequate treatment. Often, children grow normally after surgery. However, heart failure in neonatal stages and surgery can result in permanent damage to the brain and even lead to death

Who gets Vein of Galen Malformation? (Age and Sex Distribution)

  • Vein of Galen Malformation is a rare disorder that affects 1 in 25,000 every individual
  • VOGM is a congenital disorder and its manifestation can occur before or immediately after birth of the baby
  • Males and females are equally vulnerable to the disorder
  • The condition occurs worldwide, without geographic, ethnic or racial predilection

What are the Risk Factors for Vein of Galen Malformation? (Predisposing Factors)

  • Currently, the risk factors for the development of Vein of Galen Malformation are not known

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Vein of Galen Malformation? (Etiology)

The exact cause of Vein of Galen Malformation is not known. It is believed that genetic changes in the developing fetus in the first trimester can lead to this abnormality.

  • These sporadic genetic changes lead to unformed or malformed capillaries in the brain
  • The capillaries help distribute blood in the brain. They also play a role in slowing down the flow of blood. The absence of functional capillaries can lead to:
    • Intertwined clusters of blood vessels with abnormal connections between the arteries and veins
    • Blood flowing forcefully into the vein of Galen, which is a large vein carrying blood back to the heart 
  • This abnormal blood flow within and from the brain to the heart (known as vascular overload) overwhelms the organs, giving rise to the observed symptoms
  • The genetic changes in VOGM are reported to be sporadic and non-heritable

What are the Signs and Symptoms of Vein of Galen Malformation?

The signs and symptoms of Vein of Galen Malformation may include:

  • Bleeding in the brain
  • Enlarged head due to fluid accumulation in the brain (hydrocephalus); pushing of the brain tissue toward the skull
  • Seizures
  • Vascular overload leading to abnormally increased blood flow to the heart, resulting in symptoms of heart failure such as:
    • Chest pain
    • Dizziness
    • Tiredness
  • Delay in reaching developmental milestones

How is Vein of Galen Malformation Diagnosed?

The diagnosis of Vein of Galen Malformation is made by the following tools:

  • Prenatal ultrasound scan (before birth)
  • A thorough physical examination and an assessment of symptoms
  • An evaluation of the affected child’s family and medical history
  • Cerebral arteriography:
    • This test provides information on the location and characteristics of the feeding arteries and draining veins, as well as blockages
    • A thin tube is threaded up towards the brain and inserted into an artery in the groin; a dye is injected through this thin tube. The dye provides a contrast for X-ray analysis of the affected organ/area
  • Computerized tomography (CT) scans of the brain; it may include CT angiogram that may be performed with a dye injection
  • Magnetic resonance imaging (MRI) of the brain; it may include MRI angiogram with an injected dye

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Vein of Galen Malformation?

Complications of Vein of Galen Malformation can be due to the condition itself or a consequence of treatment. The complications may include:

  • Congestive heart failure
  • Stroke
  • Permanent disability

Death is known to occur from VOGM complications in some cases.

How is Vein of Galen Malformation Treated?

The treatment options for Vein of Galen Malformation may include the following:

  • Cardiac medications for individuals with complications of congestive heart failure
  • Use of ventilator for breathing difficulties; oxygen therapy for easing breathing
  • Surgery: Embolization (or blocking) of blood flow to the malformation in the brain. This reduces the flow of blood and eases vascular overload, thus improving the heart condition

How can Vein of Galen Malformation be Prevented?

Presently, there exist no guidelines or methods to prevent Vein of Galen Malformation from developing.

What is the Prognosis of Vein of Galen Malformation? (Outcomes/Resolutions)

  • The prognosis of Vein of Galen Malformation is good, if the condition is detected and treated as early as possible
  • There is a possibility of bleeding in the brain and hydrocephalus, causing permanent harm to the child
  • Babies presenting with VOGM before birth are more likely to have serious heart complications soon after birth, which can be fatal

Additional and Relevant Useful Information for Vein of Galen Malformation:

  • Dandy-Walker malformation (DWM) is a rare, congenital malformation of the brain that includes the cerebellum and the fluid filled spaces around it

The following link may help you find useful additional information on Dandy-Walker malformation:

http://www.dovemed.com/diseases-conditions/dandy-walker-malformation/

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On the Article

Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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