Haines S J, Erickson D L, Wirtschafter J D
Department of Neurosurgery, University of Minnesota, Minneapolis.
Neurosurgery. 1988 Oct;23(4):470-5. doi: 10.1227/00006123-198810000-00011.
Visual failure in osteopetrosis may result from retinal degeneration or compression of the optic nerve in the narrowed optic canals. We report the evaluation and treatment of five children with osteopetrosis whose optic nerve dysfunction seems to have been related to the latter etiology. Evaluation of visual function was carried out by means of behavioral observation and flash visual evoked responses. Of six nerves evaluated both pre- and postoperatively, the visual evoked responses seemed to improve in four. Three nerves were serially evaluated without an operation, and one showed changes consistent with maturation. One nerve was evaluated with serial postoperative evoked potentials and showed no change. We conclude that surgical decompression of the optic nerve in patients with documented optic nerve dysfunction and osteopetrosis allows improvement in visual function and is, therefore, an important consideration in the evaluation and management of patients with this disease.
骨质石化症患者的视力丧失可能是由于视网膜变性或视神经在狭窄的视神经管中受压所致。我们报告了5例骨质石化症患儿的评估与治疗情况，这些患儿的视神经功能障碍似乎与后一种病因有关。通过行为观察和闪光视觉诱发电位对视觉功能进行评估。在术前和术后评估的6条神经中，4条神经的视觉诱发电位似乎有所改善。对3条神经进行了连续评估而未进行手术，其中1条神经的变化与成熟相符。对1条神经进行了术后连续诱发电位评估，结果无变化。我们得出结论，对于有记录的视神经功能障碍和骨质石化症患者，视神经减压手术可改善视觉功能，因此，在对该病患者的评估和管理中是一个重要的考虑因素。
