Venous congestive encephalopathy related to cranial dural arteriovenous fistulas.

Cranial DAVFs present with a wide spectrum of clinical findings from pulsatile tinnitus alone to intracranial hemorrhage and NHND. The neurologic sequelae are a consequence of venous hypertension and venous congestion. DAVFs with CVR can present with or develop a VCE that can be recognized on MR imaging as a diffuse T2 hyperintensity in the deep white matter of the cerebral or cerebellar hemispheres. The T2 hyperintensity has a characteristic peripheral enhancement. The telltale sign on MR imaging is the plethora of prominent pial vessels on the surface of the brain that are the engorged cortical veins participating in the cortical venous reflux. Selective angiography is critical for the accurate assessment of the CVR. DAVFs with CVR require prompt treatment, either endovascular alone or a combination of endovascular treatment and surgery.