Giant Hypervascular Fibrous Dysplasia and Venous Aneurysm of the Central Skull Base Leading to Compressive Optic Neuropathy

We present a 24-year-old male with extensive fibrous dysplasia and an associated arteriovenous fistula with venous aneurysm causing progressive optic neuropathy. Using an endonasal endoscopic approach with nasoseptal flap reconstruction, we achieved bilateral optic nerve decompression and aneurysm removal. Pre-operative embolization mitigated bleeding risk, though transfusion requirements remained substantial. Postoperatively, imaging confirmed excellent decompression, and the patient reported subjective visual improvement. This case highlights technical challenges and multidisciplinary collaboration in managing complex skull base disease. Publication History Article published online: 27 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany