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Abstract
Optic pathway gliomas (OPG) are rare neoplasms in children with an unpredictable outcome. Because of their natural history and location there is much controversy about the optimal strategy of management of OPG. We aimed to present our experience in the management of children with optic pathway gliomas in the pediatric age group. Patients and results: Thirty seven patients (18 male/ 19 female) were included in this study, with a mean age of 5 years. OPG were chiasmatic (n=19), optic nerve (n=8), hypothalamic (n=5) and chiasmatic/hypothalamic (n=5). Six cases had NFI. Four cases were surgically debulked and 25 were biopsied (16 open, 8 stereotactic and one endoscopic) while 7 cases were totally excised. Seven out of 8 optic nerve tumors were managed by excision and the last one had no surgical intervention. Twenty six patients received chemotherapy. Histopathology revealed pilocytic (n=19), pilomyxoid (n=13), fibrillary astrocytoma (n=2) and grade I papillary-glioneuronal tumor (n=1). Patients suffered from visual affection (n=27), endocrine dysfunction (n=10) and diencephalon syndrome (n= 2). The event free survival rates at 1 and 2 years were , 88.3% and 83.4% respectively. The 2 years overall survival rate was 87.8%. (Mean follow-up duration = 21.5 months).