Genetic mutations underlying
thrombophilia are often recognized in patients with thromboembolic episodes. However, the clinical and therapeutic implications of such findings often remain unclear. We report the first case of a
dural arteriovenous fistula (DAVF) in a patient with a combined
factor II and
factor V Leiden mutation. A 40-year-old man presented with a large left temporal and intraventricular
hemorrhage. An initial angiogram showed
thrombosis of the left sigmoid sinus but no evidence of a
vascular malformation. One year after the
hemorrhage, an angiographic study showed the appearance of a right DAVF. During the follow-up period, the patient was found to harbor heterozygosity for a mutation of
factor V and a mutation of
factor II. Recognition of the patient's
thrombophilia led to prolonged oral anticoagulation
therapy to reduce the risk of a recurrent thrombotic episode. Despite the increased risk of
bleeding, the
therapy was considered justified. DAVFs may occur after
sinus thrombosis in patients with combined
factor II and
factor V mutations. This observation indicates the association of multiple hematological disorders with DAVFs in individual patients. Moreover, it raises the clinical conundrum of how to manage patients with
thrombophilia,
intracranial hemorrhage, and DAVFs.