Acute Painless Vision Loss Caused by Optic Nerve Cavernoma - A Rare Suprasellar Vascular Malformation

A 49-year-old healthy woman was evaluated in the eye emergency room for acute progressive painless vision loss in the right eye over 2 days. Six weeks prior to the visual loss, she had a routine normal ocular examination elsewhere. She had tingling sensation in the neck and arms for years. She reported intermittent hearing problem in the right ear and a few episodes of lightheadedness.

Poster 3 Acute painless vision loss caused by optic nerve cavernoma - a rare suprasellar vascular malformation Maja Kostic1, Smiljana Spasic2, Seif Tarek2, Samir Sur2, Jacques Morcos2, Sander Dubovy1, Byron Lam1 1 Bascom Palmer Eye Institute, Miami, Florida, USA, 2University of Miami, Miami, Florida, USA Introduction: A 49-year-old healthy woman was evaluated in the eye emergency room for acute progressive painless vision loss in the right eye over 2 days. Six weeks prior to the visual loss, she had a routine normal ocular examination elsewhere. She had tingling sensation in the neck and arms for years. She reported intermittent hearing problem in the right ear and a few episodes of lightheadedness. Description of Case(s): Best-corrected visual acuity was 20/300 right eye and 20/20 left eye with a right afferent pupillary defect. Confrontation visual field showed depression and dense defect inferiorly. Eye movements were full. Slit-lamp exam showed that the right optic nerve had a diffuse pallor without edema; the left was normal. Orbit and brain MRI with GAD showed a 17x12x10 mm suprasellar mass with heterogeneity in signal encompassing the right prechiasmatic optic nerve, separate from the pituitary gland and stalk. The abnormality was predominantly T1 isointense with areas of high signal and T2 heterogeneous signal. Minimal T2/FLAIR hyperintense foci in the periventricular white matter was noted. Given the heterogeneity of the mass with acute progressive vision loss, the patient was referred for surgical resection, and a diagnostic procedure was performed.Resection was performed five days after onset. Elastic van Gieson (EVG) immunohistochemistry staining showed absence of elastic layer, confirming the venous nature of the vessels. The patient's vision improved significantly from 20/300 to 20/30 at month two post-surgery. Conclusions, including unique features of the case(s): Optic nerve cavernomas (ONC) are rare and often difficult to diagnose on neuroimaging. Our case highlights the importance of including ONC in the differential diagnosis of heterogeneous lesions given treatment is challenging owing to operating in a difficult surgical field with risk of hemorrhage in an eloquent area of the brain, as well as the risk of not intervening and losing vision. References: 1. Deshmukh V, Albuquerque F, Zabramski J, Spetzler RF. Surgical management of cavernous malformations involving the cranial nerves. Neurosurgery. 53(2): 352-357; 2003. 2. Itshayek E, Perez-Sanchez X, Cohen JE, Umansky F, Spektor S. Cavernous hemangioma of the third cranial nerve: case report. Neurosurgery. 61(3): E653; 2007. 3. Matias-Guiu X, Alejo M, Sole T, Ferrer I, Noboa R, Bartumeus F. Cavernous angiomas of the cranial nerves. J Neurosurg. 73(4):620-622; 1990. 4. Ogilvy C, Pakzaban P, Lee J. Oculomotor nerve cavernous angioma in a patient with Roberts syndrome. Surg Neurol. 40(1):39-42; 1993. Keywords: Tumors, Magnetic resonance imaging Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: Maja Kostic- [e-mail], 305.399.9184 2020 Annual Meeting Syllabus | 61