Spinal dural arteriovenous fistulas presenting as intracranial subarachnoid hemorrhage: A systematic review

Abstract

BackgroundSpinal dural arteriovenous fistulas (SDAVFs) are the most common type of spinal arteriovenous malformation. Typically, these malformations present with a wide range of nonspecific symptoms indicative of thoracolumbar myelopathy. However, patients with spinal dural arteriovenous fistulas may rarely present with subarachnoid hemorrhage.MethodsA systematic review of MEDLINE and Embase databases was performed querying for cases of spinal dural arteriovenous fistulas with subarachnoid hemorrhage. Patient characteristics and outcomes investigated included spinal level of the fistula, delay of diagnosis, Hunt and Hess grade, interventions, recurrence of the fistula, and postoperative disability. Additionally, we present a unique case in which subarachnoid hemorrhage resulted from a spinal dural arteriovenous fistula that was refractory to multiple endovascular and open surgical interventions.ResultsOf 116 records identified, 45 studies were included comprising 80 patients with spinal dural arteriovenous fistula and subarachnoid hemorrhage. The most common locations of the spinal dural arteriovenous fistula were in the cervical spine (57.5%) and at the craniocervical junction (35%). Patients were treated with open surgical ligation (60.0%), endovascular embolization (22.5%), or an open surgical procedure following persistent symptoms after endovascular treatment (10.0%). Overall, the prognoses among the treated patients were favorable with only two reported (2.5%) mortalities. Rates of neurologic recovery were similar when comparing endovascular and open surgical treatment. Endovascular treatment with coil embolization of a C1-C2 spinal dural arteriovenous fistula presenting as subarachnoid hemorrhage is also described.ConclusionSpinal dural arteriovenous fistulas, particularly in the cervical spine, could be considered as a potential etiology for subarachnoid hemorrhage patients with no obvious intracranial cause. Treatment with either open surgery or embolization appears to offer a positive prognosis for both functional and angiographic outcomes.

Keywords: Onyx™; Spinal dural arteriovenous fistula; embolization; endovascular; n-butyl cyanoacrylate; subarachnoid hemorrhage.

Figure 1.

Preferred reporting items for systematic reviews and meta-analyses (PRISMA) diagram.

Figure 2.

Pre- and postoperative angiography. (A) Left VA injection, cervical, lateral: initial digital subtraction angiography (DSA) illustrating contrast extravasation of V2 at the junction of the C1–C2 vertebrae (arrow). (B) Left VA injection, cervical, lateral: day 9 DSA of the C1–C2 SDAVF displays continued delayed arterial filling of the DAVF at C2 via a muscular feeding branch from the V2/V3 region (arrow). This branch is distinct from the previously embolized branch. A coil mass is seen in the previously embolized arterial feeder with no evidence of residual contribution. (C) Left VA injection, cervical, lateral: intraoperative DSA reveals a 50% reduction in the size of the DAVF with delayed venous phase filling, predominantly from a muscular feeder from the left VA. (D) Left VA injection, cervical, lateral. Angiography performed eight months after open surgical intervention shows residual SDAVF with supply from muscular branches of the V3 segment of the left VA at C2 level, supplying a fistulous pouch. VA: vertebral artery; SDAVF: spinal dural arteriovenous fistula.