Abstract
The vein of Galen malformations are a heterogeneous group of developmental arteriovenous shunt lesions characterized by arterialization of the early embryonic choroidal venous circulation. The arteriovenous shunt lesion manifests a spectrum of age dependent clinical phenotypes each of which expresses a wide range of disease severity. Clinical presentation is determined by lesion specific, host specific and environmental factors. In recent years, patient oriented treatment strategies directed at optimizing cardiovascular and neurodevelopmental outcomes have evolved. Treatment is tailored to address specific clinical phenotypes that are differentiated according to their pathophysiology. This paper will review current approaches to the diagnosis and treatment of vein of Galen malformations patients emphasizing clinical phenotypes of the fetus, neonate and infant.
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