Optic nerve decompression for osteopetrosis in early childhood

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.