Intracranial Dural Arteriovenous Fistulae

Intracranial dural arteriovenous fistulae (dAVFs) are typified by pathological anastomoses between meningeal arteries and dural venous sinuses or cortical veins. These fistulae frequently reside within the dural leaflets surrounding a venous sinus, characteristically at the transverse-sigmoid junction but also at the cavernous sinus (CS), superior sagittal sinus, anterior cranial fossa, tentorium, and other locations. dAVFs are distinguished from their pial counterparts (eg, arteriovenous malformations) by their arterial supply from vessels that perfuse the dura mater and lack of a parenchymal nidus.1 dAVFs are rare lesions, accounting for 10% to 15% of all intracranial vascular malformations: 6% of supratentorial and 35% of infratentorial vascular malformations.2 Most frequently, dAVFs affect patients in their middle-to-later years of life (eg, 50–60 years of age).3 Less commonly, dAVFs occur in younger age groups, including children. There is neither a clear sex predilection nor genetic component to these lesions. Clinical manifestations vary widely and depend on both the hemodynamic properties and location of the fistula. Multiple classification systems have been proposed in an effort to predict which dAVFs are at high risk for new neurological events. Most of these schemes stratify risk based on angiographic appearance of the dAVFs, in particular involvement of a venous sinus and presence or absence of retrograde cortical venous drainage (CVD). Other schemes combine angiographic appearance with mode of presentation, given the impact symptoms related to intracerebral hemorrhage (ICH) and nonhemorrhagic neurological deficits (NHND) have on dAVF natural history.4–6 In the following review and synthesis, the authors describe the pathophysiology, classification, mode of presentation, imaging characteristics, natural history, and therapeutic armamentarium for intracranial dAVFs. Particular attention will be given to recent evidence that certain clinical and angiographic features of dAVFs can be used to help stratify risk of future ICH and NHND in an effort …