Objective: To evaluate the role and effect of neuro-navagation in the surgical treatment of fibrous dysplasia of the skull and to discuss the indication and advantages of transcranial optic nerve decompression are discussed.

Methods: Fifteen patients with fibrous dysplasia of skull, 6 males and 9 females, aged 10.3 (5 approximately 21), were surgically treated by transcranial approach. The location of lesion, symptoms and signs, CT characteristics, surgical approaches, and outcomes were analyzed. The postoperative outcomes of visual acuity were grouped into 5 categories: blindness, light perception, hand motion, counting fingers, and recognizing acuity chart. Improvement of visual acuity of one grade or more or increase of 0.1 by acuity chart, and improvement of visual field were defined as effective.

Results: Fibrous dysplasia of skull in children most often involved the frontal, sphenoid, and ethmoid bones and resulted in stenosis of optic canal and superior optic fissure. The most common symptoms were decreased vision, proptosis and facial asymmetry. 13 patients underwent decompression of optic canal in lateral side, and two patients in both side. All the cases had pathological diagnosis of Fibrous dysplasia of the skull. After 3-to-25-month follow-up, improvement was found in 11 eyes (65%), and no improvement was found in 6 eyes (35%). Decreased vision was not seen.

Conclusion: Transcranial optic nerve decompression is an effective treatment for decreased vision induced by Fibrous dysplasia of the skull.

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