Vein of Galen Aneurysmal Malformation presenting as Melting Brain Syndrome

Abstract

Vein of Galen aneurysmal malformations (VGAM) are congenital arterio-venous malformations in infants and fetuses which are rare and characterized by shunting of arterial flow into an enlarged cerebral vein dorsal to the tectum. These malformations present in early childhood and often present as congestive heart failure in the neonate. It is of choroidal and mural type. It can present as congestive cardiac failure in early life due to high cardiac output or show neurological manifestations due to cerebral venous hypertension. Untreated VGAM lead to chronic venous ischemia manifested by the development of subependymal atrophy with ventricular dilatation and calcification in subcortical white matter .These calcifications reflect deep hydrovenous watershed failure. These occur when the compliance of medullary veins looses its normal ventriculo-cortical gradient. We report a case of 16 month old female child presenting with vomiting, convulsions with neuroregression. MRI Brain showed prominent flow void inferior to the splenium of the corpus callosum and posterior to the third ventricle. Diffuse T2 hyperintense areas were noted involving the bilateral cerebral white matter involving fronto-temporo-parieto-occipital region with multiple foci of blooming in bilateral thalami and lentiform nuclei and in bilateral fronto-parieto-occipital subcortical and deep white matter on the GRE suggestive of chronic venous ischemia with dystrophic calcification and mild generalized cerebral atrophy suggestive of melting brain syndrome. CT Brain with cerebral angiography confirmed white matter ischemia with dystrophic calcification, VGAM and its feeders.