Compressive Optic Neuropathy from Cavernous Hemangioma

A 42-year-old man was seen for assessment of progressive blurring of vision in his left eye (OS) over several weeks. His examination showed a left optic neuropathy: visual acuity: 20/20 OD and 20/20-2 OS; pupils: 1+ left relative afferent pupillary defect; color vision: 14/14 plates correct OU. There was 2 mm proptosis of the left eye. The patient was ultimately diagnosed with a compressive optic neuropathy OS due to an intraorbital mass. The radiographic findings of his orbital mass were suggestive of an orbital cavernous hemangioma. Results from the patient's excisional biopsy confirmed the diagnosis.

Compressive optic neuropathy due to an orbital cavernous hemangioma Rahul A. Sharma, MD, MPH Valérie Biousse, MD A 42-year-old man was seen for assessment of progressive blurring of vision in his left eye (OS) over several weeks. His examination showed a left optic neuropathy: • • • • Visual acuity: 20/20 OD and 20/20-2 OS Pupils: 1+ left relative afferent pupillary defect Color vision: 14/14 plates correct OU 2 mm proptosis of the left eye Right eye (OD) Left eye (OS) Figure 1 - Color photographs centered on the optic discs showed a normal right optic nerve and an edematous left optic nerve with a peripapillary, flame-shaped hemorrhage along the superior vascular arcade. Figure 1 Flame-shaped hemorrhage Elevated, edematous optic disc with blurred margins Left eye (OS) OS OD Figure 2 - Humphrey visual fields (HVF, 24-2 SITA Fast) showed inferior arcuate visual field loss OS and a normal visual field OD. Figure 3 - Optical coherence tomography (OCT) of the retinal nerve fiber layer (RNFL) showed increased thickness of the left peripapillary RNFL, consistent with left optic disc edema (OD normal). Figure 3 - Optical coherence tomography (OCT) of the retinal nerve fiber layer (RNFL) showed increased thickness of the left peripapillary RNFL, consistent with left optic disc edema (OD normal). A B A B Figure 4 - Optical coherence tomography (OCT) of the ganglion cell layer (GCL) showed no thinning of the ganglion cell layer in either eye. Figure 5A - Axial T1-weighted imaging demonstrated an oval intraconal mass that was isointense to muscle. Figure 5B - The mass appeared hyperintense relative to muscle on axial T2-weighted imaging. Figure 5C - Post-contrast axial T1-weighted imaging with fat suppression demonstrated enhancement of the mass. The patient was diagnosed with a compressive optic neuropathy OS due to an intraorbital mass. The radiographic findings of his orbital mass were suggestive of an orbital cavernous hemangioma. Figure 6 - Results from the patient's excisional biopsy indicated a diagnosis of orbital cavernous hemangioma. A B H&E 5x, B H&E 25x. Dilated vessels lined with endothelial cells (arrows) and filled with congested red blood cells (star). Cavernous hemangioma: • Most common benign neoplasm of the orbits in adults • Commonly presents with slowly progressive proptosis • Additional clinical findings: • Retinal striae • Hyperopia (from globe compression) • Optic nerve compression (with findings of an optic neuropathy) • Increased intraocular pressure • Strabismus • Orbital imaging shows an enhancing, well-encapsulated mass • Pathology shows dilated vessels lined with endothelial cells and filled with congested red blood cells