Background Most cavernous sinus dural arteriovenous fistulas (CSDAVFs) present with benign ocular symptoms; isolated CSDAVFs with aggressive behavior are extremely rare. The treatment goal is to occlude the fistula totally. However, transarterial or venous access may not be possible because of complex angioarchitecture.

Case description A woman in her late 70s presented with progressive respiratory failure and rapid deterioration of limb muscle power. Imaging studies showed an isolated CSDAVF with exclusive venous drainage to the deep venous system leading to venous hypertension as well as ischemic changes in the brain stem, left thalamus and basal ganglia. Transvascular access of the cavernous sinus (CS) failed. The fistula was eventually occluded by transorbital direct puncture of the posterior CS through the internal carotid artery (ICA) with coils delivered into the CS. The holes in the walls of the ICA created by the puncture needle were sealed by detachable coils and the patient was discharged with mild paresis.

Conclusion Although more invasive than the transvascular route and possibly associated with the risk of loss of visual acuity and occurrence of subarachnoid hemorrhage, transorbital direct puncture of the posterior CS through the ICA is an alternative and feasible method to treat isolated aggressive CSDAVFs when access by the transvascular route fails.