When the Problem Becomes the Solution - Video

Second angiogram was performed (false-negative rate for angiographic detection of BDAVF is ~10%). Initial report was unchanged from the first one. Third review of biopsy was requested and revealed perivascular macrophages laden with hemosiderin, areas of necrosis, veins with thick hyalinized wall - all consistent with venous congestion. Consultation with interventional neuroradiologist with expertise in BDAVF was sought. All imaging was re-examined. Both angiograms demonstrated unusual tortuous cortical veins in left hemisphere in classic "pseudophlebitic pattern" seen with BDAFV. Spontaneous closure of BDAF seen in up to 10% of cases was believed to be the reason for no evidence of shunting. Hyperintense T2/FLAIR white matter lesions in left frontal lobe seen on initial MRI were compatible with ischemia secondary to longstanding BDAVF. When initial MRI/V and both angiograms were re-reviewed, definite evidence of subtle left cavernous sinus thrombosis (CST) was seen on all studies. Hypercoagulabilty work up was performed and significantly elevated level of anticardiolipin IgG antibodies was found that was likely a cause for CST. In summary, in this unique and complicated case longstanding left frontal BDAVF caused surrounding micro-ischemic changes in left frontal lobe. Subsequently, left CST developed because of hypercoagulable state caused by elevated anticardiolipin antibodies. CST caused shifting of the venous drainage from cavernous sinus to BDAFV leading to dramatically swollen left optic nerve. Compartmentalization of cavernous sinus and presence of fistula in only one compartment was the explanation for the unilateral nerve head edema without other signs of CST; it is also possible that BDAVF specifically involved left optic nerve sheath. 2.5 years after initial presentation acute venous hypertension in area supplied by BDAFV led to venous congestion, edema, and ultimately a seizure. BDAVF then spontaneously closed due to underlying predisposition to hypercoagulability, and left frontal mass with surrounding edema completely resolved several months later.