Incidence of hereditary thrombophilia in patients with cranial dural arteriovenous fistulae

Dural arteriovenous fistulas (DAVFs) can develop as consequence of prior venous sinus thrombosis. However, the prevalence of prothrombotic conditions in patients with intracranial DAVFs is unknown and there is no consensus on the indications to screen for procoagulable conditions in these patients. We performed a retrospective review of patients presenting to our institution for management of cranial DAVF. Patient and DAVF characteristics were noted, in particular the incidence of hereditary thrombophilia (HT) detected by laboratory evaluation. Respective comparisons of patients with and without HT were performed using comparative statistics. There were 165 patients presenting for management of 171 DAVFs. A large proportion of DAVF were located at a large dural sinus (42.7%), which included superior sagittal, transverse, and sigmoid sinuses. On multivariate logistic regression analysis cortical venous drainage was independently associated with hemorrhagic presentation (OR 8.23, 95% CI 1.78–38.08; p = 0.007). A HT was detected in 18 of 61 patients (29.5%) who underwent a thrombophilia work-up. Location at a large dural sinus was not more common in patients with a HT (55.0% vs 54.5%; p = 0.97). There was also no difference in the presence of cortical venous drainage (56.5% vs 38.6%; p = 0.22) or hemorrhage on presentation (15.9% vs 5.0%; p = 0.22) in patients with and without a HT. In HT were found to be highly prevalent in our cohort, though our results do not suggest their presence greatly influences DAVF pathophysiology. Screening for procoagulable states is indicated in selected patients with an intracranial DAVF.