Evaluation for Optic Pathway Glioma (OCT Should Guide Clinical Decision Making & OCT Does Not Guide Clinical Decision Making)

Optic pathway gliomas (OPGs) are low-grade neoplasms localized along the pre-cortical visual pathway. Approximately 20% of all children with neurofibromatosis type 1 (NF1) will have an OPG. Of those children diagnosed with OPGs, about 30% have NF1; although this estimate ranges from 10-75% depending on the series.(1-5) In children with NF1, these tumors are intrinsic to the anterior visual pathway axons. When OPGs are sporadic (i.e., not secondary to NF1), they are intrinsic to the axons of the optic nerve, but can be intrinsic or extrinsic to the optic chiasm or optic tract.(6) OPGs occur preferentially during the first decade of life (typically 1-8 years old) , especially in children with NF1.