Clinical Features and Treatment of Craniocervical Junction Dural Arteriovenous Fistulas

In contrast to thoracolumbar dural arteriovenous fistulas (DAVFs), the clinical features of craniovertebral junction (CVJ) DAVFs are not fully known presumably because of a pausity of data. Three cases of CVJ DAVF were reported here and a review of the literature was made to clarify their clinical features and treatment strategies. Thirty one patients with CVJ DAVFs including ours were collected. Approximately a half of them presented with subarachnoid hemorrhage (SAH). Although myelopathy remains a dominant presenting symptom, CVJ DAVFs are prone to present SAH. More than three-quarter CVJ DAVFs were fed by one or two radiculomeningeal artery at C1 or C2 level and were drained into intracranial veins via the medullary veins and the coronal venous plexus. Patterns of venous drainage appeared to be intimately correlated with clinical presentations. Interruption of the draining vein was carried out in two third of the patients and four were treated with endovascular surgery. Drainer interruption seems to be the most appropriate treatment for CVJ DAVFs, because this simple surgery reportedly provides lasting and curative treatment in most patients with spinal DAVF. There is no room for discussion in respect to surgical indication for the patients showing myelopathy. The natural history of the patients with CVJ DAVF presenting SAH has been unknown. Although surgical treatment for those patients may be justified to prevent re-bleeding, "wait and see" attitude would be appropriate for incidental CVJ DAVFs.