Optic pathway gliomas (OPGs) are low-grade astrocytomas that occur in 15-20% of children with neurofibromatosis type 1 (NF1). Although rarely life-threatening, these tumors can cause significant vision loss. Given this, the main goal of management is to reduce the risk of permanent visual loss. Half or more of NF1-OPG are indolent with minimal tumor progression or effect on vision; thus, one of the challenges in management is identifying who to treat. In addition, there is limited data on the effectiveness of chemotherapy on visual recovery or preservation. This lecture will discuss the natural history, monitoring, and management of NF1-OPG, as well as discuss emerging therapies and novel biomarkers of vision.
- Explain the approach to screening for optic pathway gliomas in children with neurofibromatosis type 1.
- Describe the natural history, treatment approach and treatment options for neurofibromatosis type 1 – associated optic pathway glioma.
- Recognize the importance of functional outcomes in assessing the success of treatment of neurofibromatosis type 1 – associated optic pathway glioma.
This seminar focuses on oncology, ophthalmology, and neurology, and was delivered at a virtual event titled, Optic Pathway Gliomas in Neurofibromatosis Type 1: Clinical features, current management and the importance of Functional Outcomes, on February 4, 2021.
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