Four-year- old child with optic nerve glioma revealed by a proptosis

Purpose Optic pathway gliomas are rare tumors accounting for 3-5% of brain tumors in children. Our case aims to understand surgical options. Methods We report the case of a 4-year-old child with a single optic pathway glioma revealed by a non esthetical proptosis and blindness. Association with NF1 was not found. The MRI showed a tortuous enlargement of the left optic nerve, the glioma extended into the optic canal with a scleral invasion. Considering those results, a treatment consisting of a double team surgery, ophthalmologists and neurosurgeons was performed with enucleation and complete section of the nerve at its optic chiasm root. Results Histology confirmed the diagnosis of glioma, as a pilocytic astrocytoma, with safety margins. Cytogenetic analysis showed an unspecific chromosome 8 deletion. Chemotherapy was avoided because of systemic toxicity and tumor localization. Conclusion Association with NF1 is classical and the incidence of optic nerve glioma is estimated at 15%. In young children the glioma can be the first manifestation of NF1 and the disease should be kept in mind. Cytogenetic mutation must be searched with surgical sample in culture. In children, surgical treatment can be preferred to chemotherapy when excision of the total tumor removal can be performed.