Review
Natural history and treatment of craniocervical junction dural arteriovenous fistulas

https://doi.org/10.1016/j.jocn.2015.05.014Get rights and content

Highlights

  • Eight patients with craniocervical junction dural arteriovenous fistulas (DAVF) evaluated at our institution were reviewed.

  • A systematic literature review of 56 studies with 119 cases was performed.

  • Prompt diagnosis and microsurgical management allows for immediate obliteration.

  • Younger age, hemorrhagic presentation, and microsurgery were predictive of good outcomes.

Abstract

Dural arteriovenous fistulas (DAVFs) located at the craniocervical junction are rare vascular malformations with distinctive features, and their natural history and the optimal treatment strategy remains unclear. We retrospectively reviewed eight patients with craniocervical junction DAVF who were evaluated at our institution between 2009 and 2012. We also conducted a MEDLINE search for all reports of craniocervical junction DAVF between 1970 and 2013, and reviewed 119 patients from 56 studies. From a total of 127 patients, 46 (37.1%) presented with myelopathy, 53 (43.1%) with subarachnoid hemorrhage (SAH), and four (3.3%) with brainstem dysfunction. SAH was typically mild, most often Hunt and Hess Grade I or II (83.3%), and associated with ascending venous drainage via the intracranial veins (p < 0.001). Higher rates of obliteration were observed after microsurgery compared to embolization. Overall, younger age (odds ratio [OR] 1.07; 95% confidence interval [CI] 1.01–1.12; p = 0.011), hemorrhagic presentation (OR 0.17; 95% CI 0.06–0.50; p = 0.001), and microsurgery (OR 0.23; 95% CI 0.08–0.6; p = 0.004) were independently predictive of good outcome at the last follow-up. Microsurgery was the only independent predictor of overall improvement at the last follow-up (OR 4.35; 95% CI 1.44–13.2; p = 0.009). Prompt diagnosis and microsurgical management, offering a greater chance of immediate obliteration, may optimize the outcomes for patients with craniocervical junction DAVF. Endovascular treatment is often not feasible due to lesion angioarchitecture, and is associated with a higher risk of lesion recanalization or recurrence. However, long term studies with newer embolic agents such as Onyx (ev3 Endovascular, Plymouth, MN, USA) are yet to be performed.

Introduction

Dural arteriovenous fistulas (DAVF) can occur in the brain or spine, and are associated with a wide range of clinical manifestations. In the spine, these lesions, which are also known as Type I spinal arteriovenous malformations (AVM), are located in the dura mater of the nerve root sleeve and are fed by radiculomedullary arteries and drain into medullary veins [1]. DAVF represent 70–80% of all spinal vascular malformations, but remain rare and underdiagnosed due to their non-specific presentation and variable time course [2]. DAVF of the craniocervical junction are especially uncommon and represent a distinct subgroup of lesions that often pose a diagnostic dilemma. Presentations can range from acute or chronic myelopathy to intracranial subarachnoid hemorrhage (SAH) to brainstem dysfunction [2]. Given their low prevalence, the natural history and the optimal treatment strategy of these lesions is unclear. Endovascular embolization and microsurgical interruption of the draining vein are both options, but these modalities have not been directly compared with respect to obliteration rates and functional outcomes [1]. This report aims to review eight institutional patients and systematically review the literature to describe the natural history and optimal treatment strategies for craniocervical junction DAVF.

Section snippets

Institutional data

A retrospective chart review was conducted of patients with craniocervical junction DAVF who were evaluated at our institution between 2009 and 2012. All study procedures were approved by our Institutional Review Board for Human Research. Craniocervical junction DAVF were defined as those lesions occurring at the level of the foramen magnum, C1, and C2. Eight patients were identified, and clinical data and diagnostic digital subtraction angiography (DSA) results were reviewed. With respect to

Institutional series

Clinical data for the eight patients evaluated at our institution for craniocervical junction DAVF are summarized in Table 1. The mean age at presentation was 62.4 ± 10.7 years (range: 44–80). Five (62.5%) of the patients presented with SAH, two with myelopathy, and one with a medullary hemorrhage. Upon angiography, all fistulae were noted to be fed by branches of the vertebral arteries (Patient 1; Fig. 2A, B). Three patients (Patients 6, 7, and 8) were not treated. One patient (Patient 7)

Clinical presentation and angioarchitecture

The natural history of craniocervical junction DAVF remains unclear. In our review, myelopathy was the most common presenting symptom. Most patients experienced progressive myelopathy over several months to years, although there were several patients with acute and rapid decline. As has been previously reported, patients can present with symptoms corresponding to any level of the spinal cord. Several reports have noted the importance of thorough diagnostic testing in patients with myelopathy of

Conclusion

Craniocervical junction DAVF can have variable clinical manifestations. SAH is typically mild and is associated with ascending venous drainage via intracranial veins with venous varices. Microsurgery is associated with a higher rate of obliteration compared to embolization. Overall, younger age, hemorrhagic presentation, and microsurgical obliteration were found to be independently predictive of good outcomes at the last clinical follow-up. These results suggest that prompt diagnosis and

Conflicts of Interest/Disclosures

Dr. Alexander Coon is a proctor for Covidien, Stryker, and Microvention. Dr. Geoffrey Colby is a consultant for Covidien and Microvention. Dr. Ali Bydon is the recipient of a research grant from DePuy Spine and serves on the clinical advisory board of MedImmune, LLC. The other authors have no financial or institutional interest in any of the drugs, materials, or devices described in this article.

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