Background: Childhood optic pathway gliomas (OPGs) are low-grade neoplasms intrinsic to the optic nerve, optic chiasm, tracts, and radiations. The management of OPGs is still a highly controversial topic among neuro-ophthalmologists.
Evidence acquisition: Authors' personal experience and literature review.
Results: This review describes our current understanding of the behavior of OPGs and discusses advances in their imaging, evaluation, and management. Patients with OPGs that progress are typically treated with chemotherapy using carboplatin and vincristine; however, newer approaches to therapy are being explored.
Conclusions: Although chemotherapy is the mainstay of treatment when indicated, multicenter collaborative studies involving oncologists and neuro-ophthalmologists, both retrospective and prospective, are still needed to establish evidence-based guidelines for the management of children with OPGs.