J Korean Neurosurg Soc.  2024 May;67(3):308-314. 10.3340/jkns.2024.0058.

Update on the Vein of Galen Aneurysmal Malformation : Disease Concept and Genetics

• Kang HS

• KMID: 2554868
• DOI: http://doi.org/10.3340/jkns.2024.0058

Abstract

Vein of Galen aneurysmal malformation is one of important pediatric arteriovenous shunt diseases, especially among neonates and infants. Here, early history of the disease identification, basic pathoanatomy with a focus on the embryonic median prosencephalic vein, classification and differential diagnoses, and recent genetic studies are reviewed.

Keyword

Vein of Galen malformations; Arteriovenous fistula; Pediatrics; Genetic testing

Figure

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  Fig. 1. Exemplary cases of vein of Galen aneurysmal malformation (VGAM). A and B : The left vertebral angiograms show a choroidal type of VGAM in a 2-week-old baby boy presenting with heart failure. The malformation has multiple feeders from the posterior choroidal arteries bilaterally and the posterior thalamoperforator, and is drained to the malformed vein (which is the persistent median prosencephalic vein) and then into the falcine sinus. C and D : The left vertebral angiograms show a mural type of VGAM in a 2-year-old boy presenting with macrocrania. There is a single-hole shunt (arrow) from the right posterior choroidal arteries.

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Reference

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