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Compared to nonaggressive cranial dural arteriovenous fistulae (cDAVF), aggressive cDAVF carries leptomeningeal venous drainage (LVD) and has approximately 15% annual risk of hemorrhagic and non-hemorrhagic aggressive neurological presentations. In terms of aggressive clinical presentations, the previous classification does not adequately differentiate the higher risk group from the lower risk group. Herein, we retrospectively collected a series of patients with aggressive cDAVF and explored the risk factors for differentiating the higher-risk group from the lower-risk group with aggressive clinical presentations. We retrospectively collected patients with aggressive cDAVF from March 2011 to March 2019. The risk of aggressive clinical presentation was recorded. Risk factors were included in the analysis for aggressive clinical presentations. From March 2011 to March 2019, 37 patients had aggressive cDAVF. Among them, 24 presented with aggressive clinical presentation (20, hemorrhagic presentation; four, non-hemorrhagic presentation). In patients presenting with hemorrhage, four patients experienced early rebleeding after diagnosis. In the univariate analysis, risk location, directness of LVD, exclusiveness of LVD, and venous strain were significantly different in patients with aggressive clinical presentation. In the multivariate analysis, exclusiveness of LVD and venous strain were observed, with a significant difference between patients with aggressive clinical presentation and those with benign clinical presentation. Among patients with angiographically aggressive cDAVFs, approximately 65% presented with aggressive clinical presentations in our series. Among all potential risk factors, patients with exclusiveness of LVD and venous strain have even higher risk and should be treated aggressively and urgently.

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