Optic chiasmatic glioma in children

We reviewed the records of 29 patients with optic nerve or chiasm glioma, or both, seen from 1955 to 1966. Sixteen patients were girls and 13 were boys. At the time of diagnosis, 14 patients were less than 2 years old. Optic atrophy was the most frequently seen physical finding, present in 26 of 29 patients. Twelve patients had diencephalic syndrome (41%). Proptosis was seen in eight. Eleven patients (38%) had associated neurofibromatosis. Pneumoencaphalogram was done on 26 patients and was abnormal in 23. The diagnosis was confirmed at surgery in 27 patients. All tumors were astrocytomas. Eighteen patients underwent radiotherapy. Surgery and radiotherapy were used as treatment for optic-chiasmatic glioma with diencephalic syndrome.