Extradural optic nerve decompression for fibrous dysplasia with a favorable visual outcome

A 10-year-old boy with progressive left visual disturbance associated with craniobasal fibrous dysplasia underwent left frontotemporal craniotomy. Dysplastic lesions of the sphenoid ridge, orbital roof, anterior clinoid, and ethmoid sinus were removed through an extradural pterional approach and the optic nerve was completely decompressed. His vision was markedly improved postoperatively. Consecutive follow-up studies for 3 years have shown no deterioration of his visual acuity. Early optic nerve decompression is highly recommended to preserve visual function in patients with craniofacial fibrous dysplasia causing visual disturbance.