1. Grand Rounds Shivani V. Reddy, M.D. University of Louisville Department of Ophthalmology and Visual Sciences

2. HISTORY o CC: “I see double” o HPI: 38 y/o WF presents with around 2 years of progressive vertical diplopia and right upper lid drooping. She also c/o decreased right peripheral vision.Denies visual acuity changes, eye pain, flashes and scotomas

3. HISTORY POHx: Refractive Error PMHx: Mitral Valve Prolapse, hypothyroidism, irritable bowel syndrome, meniere’s disease, migraine headaches syndrome, meniere’s disease, migraine headaches FAMHx: Father: glaucoma, strabismus ROS: sinusitis MEDS: synthroid, claritin, tylenol ALLERGIES: PCN

4. Exam VA TP P 20/20-2 20/20 19 18 no RAPD EOM OD: -3 upgaze restriction, -2 leftgaze restriction OS: full Hertel 15mm - (90mm)- 14mm Marginal Reflex Distance 2mm OD, 3mm OS 4→3

5. Exam OD OS LIDS/LASHES WNL WNL CONJ WNL WNL CORNEA WNL WNL IRIS WNL WNL LENS WNL WNL

6. Disc Photos OD OS Pink disc with sharp margins, normal vasculature 2+ disc edema with blurred disc margins

7. Visual Fields HVF 30-2 OD displaying enlargement of blind spot. OS WNL

8. CT ORBITS CT ORBITS W/O CONTRAST: Demonstrating right sided lesion surrounding the optic nerve with preservation of nerve architecture

9. MRI ORBITS TI – Weighted Image: Right sided homogenous enhancement surrounding but not involving lucent optic nerve T2 – Weighted Image:concentric thickening of optic nerve diameter with nonenhancing center

10. Summary o 38 y/o WF presents with 2 years of progressive vertical diplopia and subjectively decreased peripheral vision OD. Fundus exam with disc edema OD. MRI demonstrates an intraconal homogenous lesion surround the optic nerve extending to the orbital apex. VA 20/20 OU, HVF with enlargement of blind spot OD o Differential o Optic Nerve Sheath Meningioma o Optic Nerve Glioma o Orbital mets o Orbital lymphoma o Sarcoidosis

11. Treatment o Plan: Close Observation o 1 YEAR LATER o BCVA 20/30-1 o + RAPD OD, OD red saturation 60% of normal o Plan: Radiation therapy o Patient underwent intensely modulated radiation therapy of 50.4 Gy divided into 1.8 Gy fractions o Post Radiation f/u o BCVA 20/30, Red saturation 60% of normal HVF 24-2 1 year later OD: re- demonstration of enlarged blind spot

12. MRI ORBITS RECENT TI – Weighted Image with Fat suppression: Right sided homogenous enhancement surrounding but not involving non-enhancing optic nerve T1– Weighted Image with Fat Suppression: :concentric thickening of optic nerve diameter with non-enhancing center

13. Treatment o Recent exam: 2014 o BCVA 20/70 o Ishihara Plates 4/15 o + OD RAPD o Radiation Oncology not pursuing treatment at this time due to concerns for toxicity HVF 30-2 OD: stable enlarged blind spot OD

14. Optic Nerve Sheath Meningioma o Arises from meningoepithelial cells (arachnoid cap cells) lining the sheath of the intraorbital and intracanalicular portions of the optic nerve

15. Optic Nerve Sheath Meningioma o 1% - 2% of all meningiomas but 1/3 of all optic nerve tumors o 2 nd most common primary optic nerve tumor after optic nerve glioma o Epidemiology o Adults, age 40-50 o 3 times more common in women o 4-7% occur in children

16. Optic Nerve Sheath Meningioma o Classic Diagnostic Triad o Painless, slow, monocular vision loss, Optic atrophy and Optociliary shunt vessels o Disc edema seen with anterior extension of the tumor o RAPD, VF defects (enlarged blind spot, constriction) o Mild proptosis in 54% at presentation o motility defects, especially upgaze restriction

17. Imaging o MRI is gold standard. o Intense homogeneous enhancement with gadolinium o Best displayed on T1- weighted fat suppression o Concentric thickening tram track sign on axial sequences, doughnut sign on coronal o CT especially useful for evaluating the sphenoid bone and the optic nerve canal o Differs from optic nerve glioma by usual lack of calcification, and that enlargement occurs outside the nerve

19. o Observation o Patients with high functioning vision and negligible decline in visual field o When tumor is situated around the orbital apex o Close f/u of 3-6 month intervals with repeat MRI every 6-12 months suggested o VA of <20/40 and constriction of fields suggested as a marker for treatment initiation Treatment

20. o Surgery o Only indicated in patients with aggressive tumors with intracranial extension to prevent spread o Typically a blinding procedure due to shared blood supply- pial vessels are usually incorporated into the tumor o Biopsy usually undertaken in atypical presentations and when there is documented intracranial progression to the chiasm Treatment

21. o Radiation Therapy o First documented as an effective therapy in 1981 o Well defined borders of the tumor make it amenable to highly conformal therapy – delivers high dose of radiation while avoiding adjacent normal tissues o Stereotactic Fractionated Radiation Therapy (SRFT) is the most widely reported technique, usually 50 – 54 Gy given in 1.8Gy fractions Treatment

22. o Radiation Therapy o Stereotactic Radiation Surgery (SRS) with Gamma Knife has more recently come into favor o SRFT, SRS and Proton beam therapy have all shown comparable rates of tumor regression o Current literature shows that SRS has a higher risk of visual loss but provides excellent control of long term toxicity –related complications. Great treatment for patients with poor visual recovery potential Treatment

23. RADIATION TOXICITY EYELIDS20- 26 Gy CONJUNCTIVA30 Gy (conjunctivitis) LACRIMAL SYSTEM 30-45 Gy – toxicity within 5-10 years >65 Gy – toxicity within 9-10 months CORNEA30 – 50 Gy – punctate epithelial erosions 40 – 50Gy – corneal edema >60 Gy - corneal perforation IRIS>70 Gy – iritis, neovascularization LENS6.5 – 11.5 Gy – radiation to cataract time 4 years in 66% RETINA30 – 50 Gy – radiation retinopathy, 6 mo - 3 years after OPTIC NERVE>55 Gy, Fraction > 1.9 - radiation neuropathy

24. o Retrospective Study o 12 patients with monocular blindness from ONSM with tumor growth from the orbit to optic canal with extension in the intracranial space without chiasm involvement o Mean age of 43+/- 17.5 years, 9 females, 3 males o Patients underwent tumor resection and pre-chiasmatic transection of the optic nerve o Follow-up ranged over 50.6 +/- 25.7 months Clinical Neurology and Neurosurgery 115 (2013) 2426–243

25. o Results o Tumor resection achieved in 58.3% of patients o No patient developed any deterioration of contralateral vision during follow-up period o 5/5 patients with pre-op proptosis had significant improvement post-op o 67% of patients had no tumor recurrence o Of the 4 patients with recurrence, 2/4 had only undergone partial resection o Conclusion o Pre-chiasmatic transection of the optic nerve can help achieve better tumor control and preserve vision of the contralateral eye

26. 1.BSCS Book 5, Neuro-ophthalmology 2.U. Schick, U. Dott, W. Hassler. Surgical management of meningiomas involving the optic nerve sheath. J Neurosurg, 101 (2004), pp. 951–959 3.J. Shapey, H.V. Danesh-Meyer, A.H. Kaye. Diagnosis and management of optic nerve glioma. J Clin Neurosci, 18 (2011), pp. 1585–1591 4.J.Shapey, H.I. Sabin, H.V. Danesh-Meyer, A.H. Kaye. Diagnosis and management of optic nerve sheath meningiomas. J Clin Neurosci, 20 (2013), pp. 1045 – 1056. 5.B. Jeremic, S. Pitz. Primary optic nerve sheath meningioma: stereotactic fractionated radiation therapy as an emerging treatment of choice. Cancer, 110 (2007), pp. 714–722 6.T. Eng, N. Albright, G. Kuwahara, et al..Precision radiation therapy for optic nerve sheath meningiomas. Int J Radiat Oncol Biol Phys, 22 (1992), pp. 1093–1098 7.P.D. Moyer, K.C. Golnik, J. Breneman.Treatment of optic nerve sheath meningioma with three-dimensional conformal radiation. Am J Ophthlmol, 129 (2000), pp. 694–696 8.R.L. Lesser, J.P.S. Knisely, S.L. Wang, et al. Long-term response to fractionated radiotherapy of presumed optic nerve sheath meningioma. Br J Opthalmol, 94 (2010), pp. 559–563 9.Edward C. Halperin, David E. Wazer, Carlos A. Perez, and Luther W. Brady. Perez and Brady’s Principles and Practice of Radiation Oncology, 6 th edition References

27. Treatment o Patient underwent right orbitotomy with bone flap to biopsy lesion (fresh section) WHO GRADE 1 OPTIC NERVE SHEATH MENINGIOMA o PLAN: Close Observation