We reviewed the records of 218 children in whom a diagnosis of optic atrophy had been made between 1978 and 1987. A cause for the atrophy was determined for 195 patients (89%). Tumor, the most frequent cause, was found in 63 patients (29%). The most common tumor was a glioma of the anterior visual pathway; it was found in 27 patients (43% of tumors; 12% overall). The second most frequently encountered tumor, a craniopharyngioma, was found in 14 patients. Inflammation, the second most common cause of optic atrophy, occurred in 38 children (17%). Trauma caused optic atrophy in 24 patients (11%). No cause could be found for 23 patients (11%). Thirteen patients were less than 1 year of age at the time of diagnosis. Three of these patients had tumors. One was a cerebral glioblastoma, and the other two were optic gliomas. The diagnosis of optic atrophy in infancy does not imply a benign cause.
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