Abstract
Optic pathway gliomas (OPGs) are low-grade neoplasms that may be associated with profound vision loss, endocrinologic deficits, and neurologic dysfunction. OPGs occur sporadically or in association with the neurocutaneous disorder neurofibromatosis type 1 (NF1). There is ample debate regarding the most effective way for ophthalmologists to screen patients with NF1 for the presence of these tumors. Furthermore, the decision to treat and the criteria that serve as a basis for this treatment remain topics of significant discussion amongst the multidisciplinary team managing these patients including pediatric ophthalmologists, neuro-ophthalmologists, neuro-oncologists, neurosurgeons, and geneticists. Prospective natural history data are needed to develop consensus recommendations for a screening protocol for OPGs, the criteria for treatment of OPGs, and risk factors for poor visual outcomes.
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