Confirmation of communication between deep venous drainage and the vein of galen after treatment of a vein of Galen aneurysmal malformation in an infant presenting with severe pulmonary hypertension

Abstract

Vein of Galen aneurysmal malformations (VGAM) are characterized by multiple arteriovenous connections draining into a markedly enlarged median draining vein. This ectatic vein is not the vein of Galen, but its embryonic precursor, the median prosencephalic vein of Markowski. During normal development, the posterior portion of the median prosencephalic vein persists as the vein of Galen, while its anterior portion regresses in parallel with the formation of the internal cerebral veins (ICV). It has been traditionally thought that, in children with a VGAM, the deep venous system does not connect to and, a fortiori, does not drain into the ectatic median prosencephalic vein/vein of Galen. This report describes a case of successfully treated VGAM in which the drainage of an ICV into the vein of Galen was only demonstrated by follow-up MR imaging and venography. The potential implications of this finding for the management of VGAMs are discussed.

Fig 1.

Five-day-old child with cardiorespiratory failure. A, Digital subtraction angiography (DSA), left common carotoid artery, lateral view, showing enlarged anterior cerebral (arrow) and anterior choroidal (arrowhead) arteries feeding a vein of Galen aneurysmal malformations (VGAM). Note the drainage of the malformation through a falcine sinus (asterisk). B, Follow-up magnetic MR imaging 2 years after endovascular therapy; axial T2-weighted images, showing the flow void of a right internal cerebral vein (ICV) (arrowheads) draining into the shrunken vein of Galen (arrow). Note the falcine sinus (long arrow). C, Two-year follow-up MR venography, sagittal view, showing the course of the right ICV (arrowheads), its termination into the small vein of Galen (arrow), and the falcine sinus (long arrow).

Fig 1.

Five-day-old child with cardiorespiratory failure. A, Digital subtraction angiography (DSA), left common carotoid artery, lateral view, showing enlarged anterior cerebral (arrow) and anterior choroidal (arrowhead) arteries feeding a vein of Galen aneurysmal malformations (VGAM). Note the drainage of the malformation through a falcine sinus (asterisk). B, Follow-up magnetic MR imaging 2 years after endovascular therapy; axial T2-weighted images, showing the flow void of a right internal cerebral vein (ICV) (arrowheads) draining into the shrunken vein of Galen (arrow). Note the falcine sinus (long arrow). C, Two-year follow-up MR venography, sagittal view, showing the course of the right ICV (arrowheads), its termination into the small vein of Galen (arrow), and the falcine sinus (long arrow).