Harneet Randhawa OD, Navjit Sanghera OD, Leonard Messner OD ICO Primary Care/Ocular Disease Resident American Academy of Optometry Residents Day Submission

Proptosis and Decreased Vision in a Patient with a Left Sphenoid Wing

Abstract- A patient presents with complaints of gradual decreased vision and painless proptosis of the left eye. MRI imaging reveals a left sphenoid wing meningioma invading the orbit and compressing the left optic nerve.

I. Case History a. 74 yo AAF b. CC: Gradual decreased vision OS x 3 months, protruding eye OS c. POHx unremarkable OU d. PMHx (+) Hypothyroidism, type II diabetes mellitus, hypertension, hypercholesterolemia, chronic kidney disease, GERD, chronic sinusitis e. Meds- Tirosint, Apidra SoloSTAR, lisinopril, furosemide, carvedilol, digoxin, atorvastatin calcium, dexilant, Zyrtec f. H/o thyroid cancer, s/p thyroidectomy g. Hearing aid: 10+ years left ear, 2 months right ear. Patient subjectively reports hearing is worse on the left side h. Denies double vision or persistent eye pain

II. Pertinent findings a. VA sc: 20/25-2 PH NI OD, 20/30 PH NI OS b. Proptosis OS>OD c. Entrance testing i. Pupils: round, reaction 1+, no APD OU ii. EOMs: FROM, no pain OU iii. CVF: FTFC OU d. Slit lamp i. 2+ nuclear sclerosis OD, 2+ nuclear sclerosis, tr inferior anterior cortical cataract OS e. DFE i. (+) PVD OD, vitreous clear OS ii. Moderate temporal disc pallor OS iii. Macula, vessels, periphery unremarkable OU f. Hertel Exophthalmometry i. Base 100, 20 mm OD, 23 mm OS g. Additional testing i. MRI of brain and orbit 1. left sphenoid wing meningioma invading the optic canal and impinging on the left optic nerve OS 2. enlargement of the medial, inferior, superior, and lateral rectus muscles OU, asymmetrically larger lateral rectus muscle OS ii. Visual field: severe generalized depression of the entire visual field OS iii. OCT: severe temporal RNFL thinning with diffuse ganglion cell loss 360 OS iv. Fundus photos: moderate temporal pallor OS v. External photos: Proptosis OS

III. Differential Diagnosis a. Leading- Compressive Tumor b. Other- Graves Orbitopathy, Metastatic Orbital Cancer, Cavernous Carotid Fistula

IV. Diagnosis and Discussion a. Sphenoid Wing Meningioma i. Females > males ii. Higher frequency of diagnosis with advancing age iii. Most common type of sphenoid wing tumor iv. Accounts for approximately 4% of space-occupying lesions in the orbit v. 1. unilateral decreased vision due to compression of optic nerve 2. unilateral and painless on ipsilateral side of lesion 3. dysfunction and/or impairment of adjacent to tumor growth that may cause double vision 4. oculomotor dysfunction 5. Ipsilateral hearing loss secondary to compression of the Eustachian tube vi. Etiology 1. Similarities in cytological and functional studies provide evidence that meningioma cells likely stem from arachnoidal cap cells 2. at the skull base, which includes sphenoid wing meningiomas, are predominantly of epithelial origin vs. fibroblastic meningiomas found at other locations 3. Risk factors include age, hypertension, diabetes mellitus, and ionizing radiation exposure 4. Strong association between the loss of the NF2 gene on chromosome 22 or the complete loss of chromosome 22 and meningiomas a. Greater than 50% of sporadic meningiomas show loss of the either the NF2 gene or chromosome 22

b. Unique features i. Patient’s MRI revealed concomitant Graves’ Orbitopathy OU 1. Antibody-mediated reaction against the extraocular muscles causing deposition of glycosaminoglycans which results in edema and thickening of the extraocular muscles

V. Treatment/Management a. Treatment i. Surgical resection to remove the tumor 1. Studies show 81% reduction in globe protrusion and 33% reduction in visual field impairment ii. Stereotactic allows for precise radiation therapy for tumors that are adjacent to important structures, such as the optic nerve and chiasm b. Management i. Rate of tumor recurrence after total resection of meningiomas are 5% at 5 years, 10% at 10 years, and 30% at 15 years ii. Follow up care is essential to detect recurrence with continued evaluation of visual field, EOM motility, and color vision

c. References Roser F, Nakamura M, Jacobs C, Vorkapic P, Samii M. Sphenoid Wing Meningiomas with Osseous Involvement. Surgical Neurology. 2005; 64 (1): 37-43.

Heufelder MJ, Sterker I, Trantakis C, Schneider JP, Meixensberger J, Hemprich A, Frerich B. Reconstructive and Ophthalmologic Outcomes Following Resection of Spheno-Orbital Meningiomas. Opthal Plast Reconstr Surg. 2009; 25 (3): 223-226.

Lore F, Polito E, Cerase A, Bracco S, Loffredo A, Pichierri P, Talidis F. Catorid Cavernous Fistula in a Patient with Graves’ Ophthalmopathy. J Clin Endocrinol Metab. 2003 August; 88 (8): 3487-90.

Mawrin C, Chung C, Preusser M. Biology and Clinical Management Challenges in Meningioma. American Society of Clinical Oncology Educational Book. 2015; 35: e106-e115.

Tucker, Susan M., and Nancy A. Tucker. “Chapter 36 - Thyroid Orbitopathy.” Duane's Clinical Ophthalmology, 13th ed., vol. 2, Lippincott, Philadelphia, 2005, Ovid.

Rodriguez-Colon G, Bratton EM, Serracino H, Bennett JL, Hink EM. Sphenoid Wing Meningioma with Extraocular Muscle Involvement Mimicking Idiopathic Orbital Inflammation. Ophthal Plast Reconstr Surg. 2016.

VI. Conclusion a. Timely neuroimaging is essential to providing patients with the best visual prognosis for cases in which compressive optic neuropathy is suspected. b. Although Graves’ Orbitopathy has a strong association with hyperthyroidism, the condition can present in hypothyroid and euthyroid states as well. c. Due to the high likelihood of recurrence, patients with a history of meningioma require lifelong surveillance.