Dural arteriovenous fistula of the craniocervical junction along the first cervical nerve: A single-center experience and review of the literature

Abstract

BackgroundDural arteriovenous fistulas (DAVFs) of the craniocervical junction (CCJ) are relative rare lesions. Most studies of DAVFs of the CCJ included the fistulas at the foramen magnum, first cervical (C1), and second cervical (C2) level. DAVFs of the CCJ along C1 spinal nerve are rare vascular lesions with distinctive features. Our aim is to review cases of DAVFs of the CCJ along C1 spinal nerve at our institution. MethodsFrom June 2008 and December 2021. We reviewed a consecutive series of intracranial and spinal DAVFs at our institution and collected all patients harboring DAVFs of the CCJ along C1 spinal nerve. Medical charts were retrospectively reviewed regarding patient demographic data (i.e., gender and age), presenting symptoms and signs, treatment methods, and neurological outcome and complications after treatment. All image studies, including cranial computed tomography (CT) scan, cervical magnetic resonance imaging, CT angiography, and digital subtraction angiography (DSA) with rotational CT angiography were analyzed by experienced neuroradiologists. The authors also review of the literature of DAVFs of the C1 spinal nerve. ResultsThe authors identified 7 patients, including 5 men (71.4 %) and 2 women (28.6 %) with median age 54 years, range 48–72 years. Subarachnoid hemorrhage (SAH) occurred in 5 (71.4 %) patients, and progressive myelopathy in 2 (28.6 %). All fistulas except one received blood supply from the radiculomeningeal branch of the VA at C1 level. Venous aneurysms, being the source of bleeding, were detected in all fistulas with SAH. All patients except one were treated by surgical management. One fistula was treated by balloon-assisted Onyx embolization. Most patients had good neurological outcome following surgery. Complete obliteration of all fistulas treated by surgery was confirmed by follow-up DSA obtained 1 week after surgery. Two patients developed temporary pain and spasm of the trapezius muscle after the surgery. One patient resulted in poor neurological outcome and died due to sepsis and acute upper gastrointestinal bleeding one month after failed embolization. For patients with SAH, only one patient required ventriculoperitoneal shunt. ConclusionsDAVFs of the CCJ along the first spinal nerve are rare and a unique subtype of DAVFs at the CCJ. These fistulas account for 1.74 % of all intracranial and spinal DAVFs in the present study. SAH is the major manifestation of DAVFs at C1 spinal nerve that may be overlooked on routine initial DSA. Rotational CT angiography is useful for clarification of the angioarchitecture of these fistulas, including small feeding artery and venous varix. Surgical treatment by interruption of the intradural draining vein should be the treatment of choice for C1 spinal nerve DAVF