Optic Pathway Gliomas

Abstract

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.

Imaging of Optic Neuropathy and Chiasmal Syndromes

“…30 The periphery of the tumor may appear T2 hyperintense, representing arachnoidal gliomatosis (leptomeningeal involvement). 28 At times there will be significant arachnoid hyperplasia, resulting in discrepancy in size measurement on imaging and gross pathology (actual tumor size on pathology is smaller). Some ONGs may not enhance and look like cystic lesions.…”

“…29 The roles of MR imaging are to differentiate ONGs from other intraconal tumors, evaluate orbital apex/intracranial extension, assess interval growth, and screen patients with NF1 with abnormal ophthalmic findings. 28 Characteristic MR imaging findings can often preclude biopsies. On MR imaging, the tumors can be fusiform or kinked, showing T1 hypointensity, T2 isointensity to hyperintensity, and variable enhancement (Fig.…”

Pigmentary disorders of the eyes and skin

“…Studies have shown that retinal nerve fiber layer thickness inversely correlates with optic pathway gliomas and decreased vision. 37 The location of optic nerve gliomas in the noncortical visual pathway, either in the optic nerves, chiasm, tracts, and radiations, makes biopsy or surgical resection difficult due to the risk of visual loss. Chemotherapy with carboplatin and vincristine can be used to manage rapidly enlarging optic nerve gliomas.…”

Orbital Imaging

“…18,19 Most OPG in patients with NF-1 are diagnosed by age 6 years, and by age 8 years in cases of sporadic OPG. 20 Signs and symptoms at presentation include decreased visual acuity, visual field defect, proptosis, and afferent papillary defect. 20 These clinical findings may vary with the involved segments of the optic pathway.…”

“…20 Signs and symptoms at presentation include decreased visual acuity, visual field defect, proptosis, and afferent papillary defect. 20 These clinical findings may vary with the involved segments of the optic pathway. Any portion of the optic pathway may be involved.…”