Visual loss with optic atrophy is common in osteopetrosis. The efficacy of optic nerve decompression in patients with osteopetrosis is still controversial. The purpose of this study is to report that visual deterioration in osteopetrosis can be completely reversed by early optic nerve decompression. An 8-year-old female patient with osteopetrosis was found to have reduced visual acuity on routine ophthalmologic examination. Extensive optic nerve decompression was performed. It not only included unroofing the optic canal, but also drilling away bone on both sidewalls of the optic nerve. Both optic nerves were decompressed during the same surgical procedure. Progressive loss of vision associated with osteopetrosis can be prevented by optic nerve decompression in the early stage of visual deterioration. When managing patients with osteopetrosis, the importance of careful and regular assessment of visual function should be stressed.

Keywords:
Early optic nerve decompression, Osteopetrosis, Reversible visual deterioration
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© 2001 S. Karger AG, Basel
2001
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