Vein of Galen aneurysmal malformation (VGAM) is a rare congenital arteriovenous malformation of the choroid plexus situated within the roof of the third ventricle. If large, it leads to an extracardiac left-to-right shunt featuring as a high output heart failure in the neonate. In our case of VGAA4, hypoxic-ischemic brain lesions due to the steal phenomenon could be demonstrated prenatally. In a multidisciplinary clinical setting, withdrawal of active care and initiation of good end-of-life care was decided on and carried through.
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