Vein of Galen Malformation Symptoms and Causes in Children

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Vein of Galen malformation (VOGM) originates during a baby’s early prenatal development. It involves 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.

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

In VOGM, 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.

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