Background Dural arteriovenous fistulas (DAVFs) at the craniocervical junction are uncommon but clinically important abnormalities.

Objective To investigate the clinical characteristics of patients with DAVFs at the craniocervical junction and assess angiographic features associated with bleeding at presentation.

Methods We systematically reviewed the literature and searched PubMed and EMBASE for all relevant English language articles published between 1980 and 2014. The clinical presentation, angiographic characteristics, and treatment were assessed. The clinical differences between a subarachnoid hemorrhage (SAH) group and a non-SAH group were statistically examined.

Results Fifty-six patients were identified after a review of the literature (mean age 55.6 years; male to female ratio=3:1). Twenty-one patients (37.5%) presented with hemorrhage including SAH and posterior fossa hemorrhage. There was no significant difference in patient age, sex, or location of the DAVF between the SAH group and the non-SAH group. Intracranial venous drainage was significantly associated with SAH (p<0.001). The presence of a varix was significantly associated with SAH (p=0.001). Open surgery had a significantly higher efficacy of initial complete obliteration than embolization (100% vs 71.4%, p<0.01).

Conclusions DAVFs at the craniocervical junction are rare lesions, which often present with hemorrhage. Intracranial venous drainage and a venous varix are associated with increased risk of SAH. Surgical interruption of the feeding arteries or draining veins is an effective and reliable method for treating DAVFs at the craniocervical junction. Embolization is a feasible alternative to surgery in the treatment of selective DAVFs.