A multi-institutional analysis of the untreated course of cerebral dural arteriovenous fistulas

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OBJECTIVE

The rarity of cerebral dural arteriovenous fistulas (dAVFs) has precluded analysis of their natural history across large cohorts. Investigators from a considerable proportion of the few reports that do exist have evaluated heterogeneous groups of untreated and partially treated lesions. In the present study, the authors exclusively evaluated the untreated course of dAVFs across a multi-institutional data set to delineate demographic, angiographic, and natural history data.

METHODS

A multi-institutional database of dAVFs was queried for demographic and angiographic data as well as untreated disease course. After dAVFs were stratified by Djindjian type, annual nonhemorrhagic neurological deficit (NHND) and hemorrhage rates were derived, as were risk factors for each. A multivariable Cox proportional-hazards regression model was used to calculate hazard ratios.

RESULTS

Two hundred ninety-five dAVFs had at least 1 month of untreated follow-up. For 126 Type I dAVFs, there were no episodes of NHND or hemorrhage over 177 lesion-years. Respective annualized NHND and hemorrhage rates were 4.5% and 3.4% for Type II, 6.0% and 4.0% for Type III, and 4.5% and 9.1% for Type IV dAVFs. The respective annualized NHND and hemorrhage rates were 2.3% and 2.9% for asymptomatic Type II–IV dAVFs, 23.1% and 3.3% for dAVFs presenting with NHND, and 0% and 46.2% for lesions presenting with hemorrhage. On multivariate analysis, NHND presentation (HR 11.49, 95% CI 3.19–63) and leptomeningeal venous drainage (HR 5.03, 95% CI 0.42–694) were significant risk factors for NHND; hemorrhagic presentation (HR 17.67, 95% CI 2.99–117) and leptomeningeal venous drainage (HR 10.39, 95% CI 1.11–1384) were significant risk factors for hemorrhage.

CONCLUSIONS

All Type II–IV dAVFs should be considered for treatment. Given the high risk of rebleeding, lesions presenting with NHND and/or hemorrhage should be treated expediently.

ABBREVIATIONS dAVF = dural arteriovenous fistula; NHND = nonhemorrhagic neurological deficit.

Article Information

Correspondence Bradley Gross, Department of Neurological Surgery, UPMC, Ste. B-400, 200 Lothrop St., Pittsburgh, PA 15213. email: grossb2@upmc.edu.

INCLUDE WHEN CITING Published online December 15, 2017; DOI: 10.3171/2017.6.JNS171090.

Disclosures Dr. Jovin has received a grant, nonfinancial, from Fundació Ictus Malaltia Vascular and modest honoraria from Silk Road (consultant); Medtronic and Stryker Neurovascular (consultant/advisory board); and Johnson & Johnson and Neuravi (consultant). Dr. McDougall has been a consultant for Medtronic, Microvention, and Asahi Tecc.

© AANS, except where prohibited by US copyright law.

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Figures

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    Annual NHND and hemorrhage rates stratified by Djindjian type and presentation modality. Asx = asymptomatic, but also refers to any patient who did not present with either an NHND or a hemorrhage.

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    Kaplan-Meier curves for hemorrhage-free survival comparing dAVFs with and without leptomeningeal venous drainage (LVD) (A) and comparing dAVFs presenting with hemorrhage and those presenting without hemorrhage (B).

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