J Korean Ophthalmol Soc.  1999 Nov;40(11):3161-3167.

Visual Deterioration in Osteopetrosis Reversed by Early Optic Nerve Decompression

• Hwang JM
• Kim IO
• Wang KC

Abstract

Visual loss with optic atrophy is common in osteopetrosis. An 8-year-old girl with osteopetrosis showed visual deterioration presumed to be related with compressive optic neuropathy. Her grandfather, uncle on mother's side and mother have been blind because of optic atrophy associated with osteopetrosis. Bifrontal craniotomy and optic nerve decompression was performed extensively and included not only unroofing the optic canal but also drilling bone away from both walls of the optic nerve. The visual acuity improved up to 1.0 from 0.3 postoperatively. In conclusion, progressive loss of vision associated with osteopetrosis could be prevented by optic nerve decompression in the early stage of optic neuropathy. In management of patients with osteopetrosis, importance of careful and regular assessment of visual function should be stressed.

Keyword

Optic nerve decompression; Osteopetrosis; Reversible visual deterioration

MeSH Terms

Child
Craniotomy
Decompression*
Female
Humans
Mothers
Optic Atrophy
Optic Nerve Diseases
Optic Nerve*
Osteopetrosis*
Visual Acuity