High-flow Neonatal Macrocerebral Arteriovenous Fistulas in Hereditary Hemorrhagic Telangiectasia

Although some cases of vein of Galen aneurysmal malformation (VGAM) present initial clinical symptoms such as cardiopulmonary disturbance in the neonatal period, pial arteriovenous fistula is very seldom present as a clinical symptom immediately after birth. A neonatal patient, the first-born to his family, presented with tachypnea postpartum. This baby had a family history of hereditary hemorrhagic telangiectasia. A cerebral MR image revealed multiple macrocerebral arteriovenous fistulas (MCAVFs), resulting in a large partially thrombosed venous pouch within the cerebral cortex. Trans-arterial embolizations of the main two fistulas were performed using N-butyl cyanoacrylate (NBCA) with tantalum powder six months after birth. Post-embolization angiography confirmed the obliteration of the fistulas and magnetic resonance imaging (MRI) revealed thrombosis and reduction in size of the venous component. His tachypnea disappeared completely. There were no neurological complications due to the treatment. The prognosis of multiple MCAVFs mainly depends on the presence of medullar signs and symptoms and a delay before treatment. Pure glue endovascular intervention, as used in our case, is considered to be the first therapeutic choice to decrease the risk of neurological consequences.