Visual Deterioration in Osteopetrosis Reversed by Early Optic Nerve Decompression.

Hwang, J M; Kim, I O; Wang, K C

Original Article

Journal of the Korean Ophthalmological Society 1999;40(11):3161-3167.
Published online November 1, 1999.

Visual Deterioration in Osteopetrosis Reversed by Early Optic Nerve Decompression.

¹Department of Ophthalmology, Seoul Municipal Boramae Hospital.
²Department of Radiology, College of Medicine, Seoul National University, Seoul, Korea.

골화석증에서 조기 시신경감압술 후 시력회복

황정민(Jeong Min Hwang),김인원(In One Kim),왕규창(Kyu Chang Wang)

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.

Key Words: Optic nerve decompression;Osteopetrosis;Reversible visual deterioration