Multiple synchronous intracranial dural arteriovenous fistulas.

Background: Intracranial multiple dural arteriovenous fistulas (DAVFs) are rare, with most reports limited to small case series and basic descriptive analyses. To better understand this condition-often linked to lower cure rates and higher progression risk-we conducted a comparative analysis of their angiographic characteristics, clinical presentations, and outcomes.

Methods: Data were retrospectively collected from the Dural Arteriovenous Fistula Research and Management in China (DREAM-INI) database. Lesions were classified as multifocal or diffuse based on angiographic features, and as progressive or non-progressive based on follow-up angiographic findings. We compared these subtypes as well as multiple versus single DAVFs, and further investigated the predictors of disease progression in patients with multiple DAVFs.

Results: A total of 90 cases of multiple synchronous DAVFs were included. Compared to the multifocal type, diffuse-type patients were younger (P = 0.002), and more frequently exhibited sinus occlusion/stenosis (P = 0.019), sinus-type DAVF (P < 0.001), pial artery supply (P = 0.009), venous congestion (P = 0.030), and lower complete obliteration rates (P = 0.001). Progressive DAVFs were associated with younger age (P = 0.001), sinus-type DAVF (P = 0.020), higher rates of diffuse-type lesions (P < 0.001), pial artery supply (P = 0.037), deep venous drainage (P = 0.032), and venous congestion (P = 0.005). Among the 14 treatment-related complications, 8 (57.1%) were associated with pial artery embolization. Compared to single DAVFs, patients with multiple DAVFs had a significantly lower rate of good outcomes (P < 0.001) and a higher incidence of fistula-related death (P = 0.001). On multivariate analysis, only younger age remained a significant predictor of progression (OR 2.86; 95% CI 1.02-7.99; P = 0.045).

Conclusions: Diffuse and progressive types often occur in younger patients and are associated with sinus-type fistulas and venous hypertension-related angioarchitectural features. Disease progression may involve enhanced neoangiogenesis. Treatment of refractory DAVFs should prioritize reducing venous hypertension and improving cerebral hemodynamics, rather than solely pursuing complete obliteration.