Vein of Galen Malformations : Figure 1

Classical presentation of a choroidal-type vein of Galen malformation: neonate presents with severe congestive heart failure difficult to control medically and has a loud cranial bruit. A, Transcranial color Doppler ultrasonography demonstrates an abnormally large high-flow midline vascular structure (white arrow). B, CT brain scan confirms the presence of an enlarged midline vascular structure that is highly suspicious for a vein of Galen malformation in the neonate with high-output cardiac failure (white arrow). C, a combination of trans-arterial and trans-venous techniques are used to completely occlude this type of congenital fistula with aneurysmal dilatation of the median prosencephalic vein of Markowski (black arrows). D, Because there was no pre-existing brain atrophy, this child made a complete and full recovery following treatment and remains neurologically normal several years following curative embolization.

Two year old boy with increased head circumference (increasing head size) and Parinaud’s Syndrome (paralysis of upward gaze due to compression of the brainstem tectum – base of brain). A and B, Sagittal T1-weighted and axial T2-weighted images reveal marked enlargement of a central venous structure represented the dilated median prosencephalic vein of Markowski with compression of the dorsal midbrain and cerebral aqueduct with resultant hydrocephalus.C, Left vertebral artery injection, frontal projection in the arterial phase, demonstrates a mural-type vein of Galen malformation supplied by the collicular arteries through a single hole fistula (curved arrow).D, Left vertebral injection, lateral projection in the late venous phase, shows to advantage the dilated prosencephalic vein of Markowski draining to a persistent falcine sinus. In these cases, the straight sinus is often absent, and it has been proposed that intra-uterine thrombosis of the straight sinus results in the vein of Galen malformation.E, Left vertebral injection, frontal projection in the arterial phase following occlusion of the right and left collicular arteries, show complete occlusion of the malformation. The patient made an uneventful recovery and is now neurologically normal.