Rete Mirabile Associated with Pial Arteriovenous Fistula: Imaging Features with Literature Review

A rete mirabile is a vascular network of intercommunicating small arteries or arterioles that replace the definitive adult artery supplying the brain. It supplies the brain in lower mammals but is not seen in normal human embryological development. A 26-year-old man presented with worsening tinnitus that was interfering with his sleep. On CT and digital subtraction angiography he was found to have a temporal lobe pial arteriovenous fistula with bilateral carotid and vertebral rete mirabile. The patient was offered open surgical and endovascular treatment options for pial arteriovenous fistula but he refused both and opted for conservative medical management. At 6-month follow-up he continued to have pulsatile tinnitus but was otherwise neurologically normal. We present the first described association of rete mirabile with pial arteriovenous fistula and discuss its clinical presentation and imaging features, with a review of the literature for bilateral carotid and vertebral rete mirabile.