A Pial Arteriovenous Fistula in Infancy as the Presenting Manifestation of Hereditary Hemorrhagic Telangiectasia.

Abstract

Pial arteriovenous fistulas (PAVFs) are rare, accounting for 1.6%-4.7% of all intracranial vascular malformations. Often diagnosed in childhood, about 30% are associated with hereditary hemorrhagic telangiectasia. A case of PAVF diagnosed soon after birth and given cerebrovascular therapy 4 months after birth is reported. The patient presented with heart failure immediately after birth. Ultrasonography of the head showed abnormal blood flow in the brain. On digital subtraction angiography performed 4 months after birth, a PAVF with a dural feeder shunt and a giant varix at the posterior temporal part was confirmed. After transarterial embolization (TAE), shunt blood flow disappeared. New shunt flow from the right posterior cerebral artery into the varix was confirmed by magnetic resonance imaging 3 months after the operation. A second TAE procedure using a liquid embolic material was performed and confirmed the complete disappearance of the shunt. This report describes a case of infant PAVF with heart failure, a giant varix, hydrocephalus, and intraventricular hemorrhage treated by TAE using platinum coils and liquid embolic material.