Variations and management for patients with craniocervical junction arteriovenous fistulas: Comparison of dural, radicular, and epidural arteriovenous fistulas

Abstract

Background: Craniocervical junction arteriovenous fistulas (CCJAVFs) are known to be rare, but variations and clinical behaviors remain controversial. Methods: A total of 11 CCJAVF patients (M: F=9:2, age 54–77 years) were investigated. Based on the radiological and intraoperative findings, they were categorized into three types: dural AVF (DAVF), radicular AVF (RAVF), and epidural AVF (EDAVF). Results: There were four symptomatic patients (subarachnoid hemorrhage in two, myelopathy in one, and tinnitus in one) and seven asymptomatic patients in whom coincidental CCJAVFs were discovered on imaging studies for other vascular diseases (arteriovenous malformation in one, intracranial DAVF in two, ruptured cerebral aneurysm in two, and carotid artery stenosis in two). Of these 11 patients, 2 (18.2%) had multiple CCJAVFs. Of 14 lesions, the diagnoses were DAVF in 5, RAVF in 3, and EDAVF in 6 (C1–C2 level ratio =5:0, 2:1, 3:3). Patients with DAVF/RAVF in four lesions with intradural venous reflux underwent surgery, although an RAVF remained in one lesion after embolization/radiation. Since all six EDAVFs, two DAVFs, and one RAVF had neither feeder aneurysms nor significant symptoms, no treatment was provided; of these nine lesions, one DAVF and one RAVF remained unchanged, whereas six EDAVFs showed spontaneous obliteration within a year. Unfortunately, however, one DAVF bled before elective surgery. Conclusion: CCJAVFs have many variations of shunting site, angioarchitecture, and multiplicity, and they were frequently associated with coincidental vascular lesions. For symptomatic DAVF/RAVF lesions with intradural drainage, surgery is preferred, whereas asymptomatic EDAVFs without dangerous drainage may obliterate during their natural course.

Efficacy of intraarterial indocyanine green videoangiography in surgery for arteriovenous fistula at the craniocervical junction in a hybrid operating room: illustrative cases

“… 6–8 Recent developments in various neuroradiological modalities and accumulation of surgical experience have led to the identification of radicular AVF on the spinal nerves located between the spinal cord and dura in which radiculomeningeal and pial feeders connect with the radicular vein at the same fistulous point. 9 , 10 In Case 2 of our report, radicular AVF was ruled out based on the lack of a fistulous point or feeders from the radiculomeningeal artery on the nerve root demonstrated by intraarterial ICG videoangiography. Hiramatsu et al classified CCJ AVF into five subgroups.…”

Subarachnoid hemorrhage due to a craniocervical junction arteriovenous fistula associated with thrombus formation in the internal jugular vein: illustrative case

“…A craniocervical junction is a rare AVF lesion that accounts for 1% to 2% of intracranial or spinal AVFs 1 , 4 and mainly occurs in middle-aged men. 1 , 2 , 6 , 7 CCJAVFs have a wide range of clinical presentations, including acute SAH, 8–11 myelopathy, 12 intramedullary hemorrhage, 2 brainstem dysfunction, 6 , 13–15 and radiculopathy. 3 Our patient had respiratory disorders and tetraparesis after admission and completely recovered after treatment.…”

“… 23 , 24 Several studies have suggested that venous hypertension may be associated with CCJAVF. 2 , 17 Another possible etiology is thrombosis or congestion of the internal dural vein due to bone fracture or postoperative fibrosis causing an abnormal shunt. 22 In the present case, SAH was probably caused by ascending intracranial drainage, but the AVF mainly drained into the epidural plexus.…”

“…1,3,4,11,17 In previous reports, risk factors for bleeding, including intracranial drainage, varix, feeder aneurysm, and feeders from the anterior spinal arteries, have been mentioned. [1][2][3]11,16 Epidural drainage has been reported to infrequently cause a hemorrhage. 2 The treatment for CCJAVF remains controversial.…”

“…A craniocervical junction arteriovenous fistula (CCJAVF) is a rare vascular malformation with various draining patterns. [1][2][3][4] Because of its rarity, the etiology remains uncertain, and the clinical course is sometimes unpredictable. 5,6 Herein we report a case of a subarachnoid hemorrhage (SAH) caused by CCJAVF associated with internal jugular vein (IJV) occlusion.…”