Optic nerve sheath fenestration for an isolated optic nerve glioma.

The treatment modalities for neurofibromatosis type 1-associated optic gliomas include chemotherapy, radiation therapy, and surgical excision. The current recommendation is to consider treatment for an optic nerve glioma only if there is clear evidence of either ophthalmologic or radiographic progression with significant visual dysfunction. We report a case of a child with neurofibromatosis type 1 and an isolated optic nerve glioma with documented progression and visual loss in which clinical signs improved and visual deterioration stabilized after optic nerve sheath fenestration.