INTRODUCTION	Section 2 of 10
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Background: The vein of Galen is located under the cerebral hemispheres and drains the anterior and central regions of the brain into the sinuses of the posterior cerebral fossa. Aneurysmal malformations of the vein of Galen (VGAM) typically result in high-output congestive heart failure or may present with developmental delay, hydrocephalus, and seizures.

Pathophysiology: VGAM results from an aneurysmal malformation with an arteriovenous shunting of blood. The congenital malformation develops during weeks 6-11 of fetal development as a persistent embryonic prosencephalic vein of Markowski; thus, VGAM is actually a misnomer. The vein of Markowski actually drains into the vein of Galen.

VGAM usually causes high-output heart failure in the newborn resulting from the decreased resistance and high blood flow in the lesion. Associated findings include cerebral ischemic changes such as strokes or steal phenomena that result in progressive hemiparesis. Hemorrhage from the malformation can occur, although this is not a common finding. Finally, the malformation may result in mass effects, causing progressive neurological impairment. Alternatively, the malformation may cause obstruction of the cerebrospinal fluid (CSF) outflow and result in hydrocephalus.

Frequency:

• In the US: Unknown

• Internationally: Unknown

Mortality/Morbidity:

• Infants often die if the high-output congestive heart failure is the presenting feature.

• Macrocephaly usually improves following shunting for hydrocephalus.

Race: VGAM occurs in all races.

Sex: Boys and girls are affected equally.

Age: VGAM is a congenital malformation; therefore, it may present at birth or in early childhood.

	CLINICAL	Section 3 of 10
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Pictures Bibliography

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:

• Congestive heart failure

• Neonates may present with tachypnea, respiratory distress, and cyanosis.

• They often require ventilatory support and institution of aggressive management of heart failure.

• Hydrocephalus

• Hydrocephalus may be the presenting feature in older infants.

• A cause should be sought in neonates with macrocephaly.

• Infants may have hydrocephalus, in which case prominent scalp veins or “sunset” eye findings are noted.

• 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.