Arteriovenous fistulas (AVFs) are direct pathologic arteriovenous shunts representing 10-15% of all cerebrovascular malformations with AV shunting. The prototypical AVF is an acquired lesion between transosseous-extracranial and/or meningeal arteries into the wall of a thrombosed dural venous sinus compromising a dural AVF (DAVF) and resulting in hypertensive venous congestion and venopathy, however pathologic arteriovenous fistulous connections may exist along any dural margin, sinus, or venous tributary.
DAVFs are distinguished from pial-parenchymal arteriovenous malformations by the predominance of dural arterial supply and the absence of a parenchymal nidus, however AVMs may have pial or dural fistulous components. The majority of DAVFs present in adulthood and are most commonly located at the transverse-sigmoid sinus wall. Clinical presentation and imaging findings are highly variable depending on anatomic site, degree of AV shunting, and venous reflux.
Transverse-sigmoid sinus DAVFs typically present with pulsatile tinnitus. DAVFs may present with encephalopathic symptoms secondary to venous hypertension, ischemia, and/or thrombosis. DAVFs with deep/inferior drainage to the petrosal sinuses and perimedullary venous plexus may result in progressive myelopathy and central respiratory failure.
Congenital arteriovenous fistulas, including DAVF, Vein of Galen Aneurysmal Malformations (VGAM) of the subarachnoid space, and non-galenic pial AVF (pAVF) encountered in the pediatric population, have unique developmental and pathophysiologic characteristics and tend to be very rare. Like AVMs, underlying parenchymal abnormalities have been hypothesized to result from vascular steal phenomenon and venous congestion. Infants with large congenital DAVF, VGAM, and non-galenic pAVFs can present with congestive heart failure (early) and developmental delay or increased head circumference (late).
Carotid Cavernous Fistulas (CCF), included in this category, represent pathologic shunts between the cavernous ICA and the cavernous sinus, resulting in unique symptomology typically with pulsatile exophthalmos and cranial neuropathies of III, IV, and VI.
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