Giant calcified thrombosed varices secondary to a pial arteriovenous fistula associated with hereditary hemorrhagic telangiectasia

Abstract

A 28-year-old woman presented with an unusual case of giant thrombosed varix with calcified walls that had mass effects secondary to a pial single-channel arteriovenous fistula (AVF) associated with hereditary hemorrhagic telangiectasia (HHT). She consulted our hospital for chronic headache. She had been diagnosed with HHT based on genetic testing when her 3-year-old son presented with subarachnoid hemorrhage due to spinal AVF. Imaging studies revealed pial single-channel AVF with multiple varices. The varices in the right frontal lobe were over 6 cm in diameter and had laminar thromboses and calcified walls. Because of the mass effect, direct surgical flow disconnection was performed followed by removal of the varices using an internal decompression technique. Postoperatively, the patient was discharged with no neurological symptoms and no longer suffered chronic headache. Intracerebral varices are occasionally associated with high-flow AVF, and usually treated by interrupting the feeding arteries leaving the varices intact. This extremely rare case of intracerebral giant thrombosed varices with calcified wall and mass effect indicates that surgical removal of varices should be considered.