History
Direct communication between a cerebral artery and a cerebral vein results from a congenital vascular malformation. The vein of Galen abnormality is the most frequent arteriovenous malformation in neonates.
It commonly presents in the neonatal period, although it may present later, in early childhood. Typically, in the neonatal period, VGAM presents with congestive heart failure, a cranial bruit, and marked carotid pulses.
Physical
Physical findings include heart failure and hydrocephalus.
Congestive heart failure
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Neonates may present with tachypnea, respiratory distress, and cyanosis.
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They often require ventilatory support and institution of aggressive management of heart failure.
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Hydrocephalus may be the presenting feature in older infants.
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A cause should be sought in neonates with macrocephaly.
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Infants may have hydrocephalus, in which case prominent scalp veins or "sunset" eye findings are noted.
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Developmental delay: Signs of hydrocephalus and congestive heart failure should be looked for in infants with developmental delay.
In early childhood, symptoms include headache, convulsive seizures, hydrocephalus, and cardiac failure.
Causes
Although any vessel may be affected, the vein of Galen is the most frequently affected. Congenital malformation develops during weeks 6-11 of fetal development as a persistent embryonic prosencephalic vein of Markowski.
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Cerebral MRI showing large flow void in the central region with enlarged straight sinus.
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Coronal MRI of head showing large central vein of Galen malformation with moderate degree of hydrocephalus.
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Cranial MRI showing flow void in the sagittal plain and drainage to the straight and transverse sinuses.
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Sagittal cerebral MRI with gadolinium showing the relationship of a vein of Galen malformation to the corpus callosum.
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MRI venogram showing vein of Galen malformation with draining veins.
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Venogram showing the draining vasculature for the vein of Galen malformation.
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Skull radiograph showing coils that have been placed during an intravascular embolization of a vein of Galen malformation. Note the ventriculoperitoneal shunt catheter in the anterior head region to relieve hydrocephalus.
