Intracranial pial arteriovenous fistulas.

Intracranial pial arteriovenous fistula (AVF) is a rare cerebrovascular lesion that has only recently been recognized as a distinct pathological entity. A 41-year-old woman (Patient 1) presented with the sudden development of an altered mental state. Brain CT showed an acute subdural hematoma. A red sylvian vein was found intraoperatively. A pial AVF was revealed on postoperative angiography, and surgical disconnection of the AVF was performed. A 10-year-old boy (Patient 2) presented with a 10-day history of paraparesis and urinary incontinence. Brain, spinal MRI and angiography revealed an intracranial pial AVF and a spinal perimedullary AVF. Endovascular embolization was performed for both lesions. The AVFs were completely obliterated in both patients. On follow-up, patient 1 reported having no difficulty in performing activities of daily living. Patient 2 is currently able to walk without assistance and voids into a diaper. Intracranial pial AVF is a rare disease entity that can be treated with surgical disconnection or endovascular embolization. It is important for the appropriate treatment strategy to be selected on the basis of patientspecific and lesion-specific factors in order to achieve good outcomes.