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Show Poster 205 Three types of optic disc edema in optic nerve sheath meningioma Susiani Intan1, Shannon Beres1, Heather Moss1, Joyce Liao1 1 Byers Eye Institute at Stanford, Palo Alto, California, USA Introduction: ONSM (optic nerve sheath meningioma) accounts for approximately 2% of all orbital tumors and represents around 1–2% of all meningiomas. Classical triad of progressive visual loss, optic nerve atrophy, and presence of optociliary shunt vessels is suggestive of an ONSM. Treatment of ONSM are radiation therapy, surgery, and observation. Description of Case(s): Case 1 A 62-year-old male presented with progressive left vision loss over 3-4 years found to have ONSM. He completed total of 50.4 Gy in 28 fractions radiation therapy. Optic disc edema noted on his initially presentation which improved after radiation therapy. Another optic disc edema was noted 4 months after radiation treatment with worsening vision. He received corticosteroid with improvement of optic disc edema but not his vision. Case 2 A 46-year-old woman presented with right optic atrophy with progressive vision loss found to have WHO grade 1 right ONSM. She received both surgical and radiation treatment. She completed total 50.4 Gy in 28 fractions radiation therapy. 1 year after radiation she presented with worsening vision on the right eye. OCT with increase average RNFL suggested right optic nerve edema. She was treated with corticosteroid with rapid reduction of swelling and slight visual improvement. Conclusions, including unique features of the case(s): 3 different periods of optic disc edema noted on these 2 cases and ONSM treated with radiation: 1. Optic disc edema at presentation prior to treatment due to compression of the optic nerve causing impaired axonal transport 2. Optic disc edema shortly after radiation treatment: a. Acute (during treatment) due to radiation endothelial damage leads to blood brain barrier breakdown and Cytokine release b. Early delayed (2-4 months after radiation therapy) due to glial dysfunction leads to demyelination gliosis & inflammation 3. Optic disc edema one year after radiation due to late delayed radiation toxicity & radiation necrosis. References: Moster ML. Detection and treatment of optic nerve sheath meningioma. Curr Neurol Neurosci Rep. 2005 Sep;5(5):36775. doi: 10.1007/s11910-005-0060-x. Douglas VP, Douglas KAA, Cestari DM. Optic nerve sheath meningioma. Curr Opin Ophthalmol. 2020 Nov;31(6):455-461. doi: 10.1097/ICU.0000000000000700. Furuse M, Nonoguchi N, Kawabata S, Miyatake S, Kuroiwa T. Delayed brain radiation necrosis: pathological review and new molecular targets for treatment. Med Mol Morphol. 2015 Dec;48(4):183-90. doi: 10.1007/s00795-015-0123-2. Miller NR. New concepts in the diagnosis and management of optic nerve sheath meningioma. J Neuroophthalmol. 2006 Sep;26(3):200-8. doi: 10.1097/01.wno.0000235569.19131.ac. Lessell S, Kim JW, Hatton MP, Stemmer-Rachamimov A, Thiagalingham S, Rubin PA. Clinical without histopathological manifestations of inflammation in a patient with primary intraorbital optic nerve sheath meningioma. J Neuroophthalmol. 2007 Jun;27(2):104-6. doi: 10.1097/WNO.0b013e318064e495. Keywords: chemotherapy and radiation injury, optic neuropathy, orbit/ocular pathology, tumors, diagnostic tests (ERG, VER, OCT, HRT, mfERG, etc) Financial Disclosures: The authors had no disclosures. Grant Support: NIH P30 026877: Unrestricted grant research to Prevent Blindness Contact Information: None provided. 2021 Annual Meeting Syllabus | 205 |
