Non-Galenic Pial Arteriovenous Fistula: Case Report of a Rare High-Flow Vascular Anomaly in a Neonate

Non-Galenic pial arteriovenous fistulas (NGPAVFs) are very rare in neonates and pose serious risk for morbidity and mortality. NGPAVFs are characterized by a direct arteriovenous connection between a pial artery and a cortical vein without an intervening capillary bed. Lack of a nidus differentiates an arteriovenous fistula from an arteriovenous malformation. In neonates, the clinical presentation and prognosis of NGPAVF are similar to those of the more common vein of Galen aneurysmal malformation, from which it must be differentiated. We report a case of a high-flow, multi-hole variant of a NGPAVF presenting with high-output heart failure with an imaging-focused description. This report discusses how the imaging appearance of an NGPAVF compares with that of other high-flow malformations and includes a brief literature review.

Learning Objective: NGPAVF, a rare cerebrovascular malformation, should be included in the differential diagnosis of high-flow intracranial malformations in neonates with high-output cardiac failure. Alternative diagnoses include vein of Galen malformation, dural arteriovenous fistula, and cortical arteriovenous malformation, among other entities.