Vein of Galen Malformation

Stanley Hoang, B.S.; Omar Choudhri, M.D.; Michael Edwards, M.D.; Raphael Guzman, M.D.

Disclosures

Neurosurg Focus. 2009;27(5):E8 

In This Article

Conclusions

A VGM is a congenital vascular malformation consisting of multiple arteriovenous shunts draining into the median prosencephalic vein of Markowski, which is not actually the vein of Galen itself, but rather a persistent embryonic structure that precedes the vein of Galen. Classically, VGM presents in the neonatal period with cardiac output failure and in severe cases with diffuse brain destruction referred to as melting brain. In infancy the main findings are hydrocephalus and developmental delays, and in childhood intracerebral and subarachnoid hemorrhage. While the prognosis of VGM has been especially grim in the past, with the mortality rate approaching 100% in some cases, many studies have shown that endovascular embolization is highly effective in improving the neurological outcome of patients with VGM. The timing of embolization is critical for the best possible outcome, with emergency treatment of neonates suffering acute heart and renal failure and delayed treatment if not suffering acute distress, optimally at 5–6 months of age. When CSF ventricular shunting is necessary to alleviate hydrocephalus, it should be performed after the embolization procedure.[2] The use of a 21-point scale score based on cardiac, cerebral, hepatic, respiratory, and renal function can be used to guide therapeutic decisions.[31] Endovascular therapy together with a comprehensive, multidisciplinary approach in the intensive care units has now emerged as the optimal approach for improving the poor prognosis of patients with VGM.

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