Object: Cranial dural arteriovenous fistulas (DAVFs) can be classified into benign or aggressive, based on their patterns of venous drainage. A benign condition requires the absence of cortical venous drainage (CVD). The clinical and angiographic features of a consecutive single-center group of 117 patients harboring benign cranial DAVFs were evaluated over time to validate the behavior and appropriate management of these lesions.
Methods: At the initial assessment four patients were asymptomatic. Two infants presented with congestive heart failure. All other patients presented with other benign symptoms: chronic headache, bruit, or orbital phenomena. Observational management was instituted in 73 patients (62%). Intolerable bruit or ophthalmological sequelae were deemed indications for palliative embolization in 43 patients and surgical treatment in one patient. A median follow-up period of 27.9 months (range 1 month-17.5 years) was available in 112 patients (95.7%), among whom repeated angiography was performed in 50. Overall, observational and palliative management resulted in a benign and tolerable level of disease in 110 (98.2%) of 112 cases. In two cases managed conservatively CVD developed. In both of these cases the conversion from benign to aggressive DAVF was associated with spontaneous progressive thrombosis of venous outlets.
Conclusions: The disease course of a cranial DAVF without CVD is indeed benign, obviating the need for a cure of these lesions. Symptoms are well tolerated with either observation or palliative treatment. After a long-term follow-up review of 68 patients, this conservative management resulted in a benign and tolerable level of disease in 98.5% of cases. It is noteworthy, however, that a benign DAVF carries a 2% risk of developing CVD, mandating close clinical follow-up review in such cases and renewed radiological evaluation in response to any deterioration in the patient's condition.