Optic Disc Melanocytoma Associated With Optic Nerve Head Edema, Macular Star, and Venous Stasis Retinopathy

Abstract

Abstract: : Purpose:To report a rare case of melanocytoma with optic nerve head edema, venous stasis retinopathy, and a macular star with good visual recovery. Methods:In addition to full ophthalmic examination, the patient also underwent fluorescein angiography, and color fundus photography. Results:A 41 year–old Nigerian female with a past medical history significant only for hypertension presented with a two week onset of decreased vision in her left eye. She denied animal exposure or symptoms associated with a viral prodrome. Visual acuity was 20/20 in her right eye, counting fingers with an APD in the left eye. Intraocular pressure was 18mm mg OU. Anterior segment and right fundus exam were normal. Funduscopic exam of the left eye revealed frank optic nerve head edema with tortuous, engorged, posterior pole vessels, mild scattered nerve fiber layer dot and blot hemorrhages noted in all four quadrants, and a macular star. Flourescein angiography revealed a delayed arteriovenous transit time, mild optic nerve leakage, and blocked hypoflouresence corresponding to the intraretinal hemorrhages. Medical work–up including evaluation for sarcoidosis, syphilis, lyme disease, and bartonella were negative. At one month follow–up the patient had decreased optic nerve head edema, less macular exudation, and improved visual acuity to 20/200. A feathery pigmented lesion of the superior optic nerve extending into the nerve fiber layer consistent with a melanocytoma was noted. Over the next 4 months, she was noted to have visual improvement to the 20/60 level with macular exudate resolution. Conclusions:While melanocytoma of the optic nerve is most often a benign lesion of little visual significance, it has been previously documented to cause acute visual loss leading to permanent visual sequelae presumably due to vascular occlusion. In 2001, Shields et al reported a case of a 35 year–old healthy male of African descent with histopathologic documentation of a combined central retinal artery and vein occlusion secondary to necrotic melanocytoma of the optic nerve. Zimmerman also reported similar findings in a case of central retinal artery obstruction. To our knowledge there have been five cases of melanocytoma associated with visual loss reported in the literature. Central vascular occlusion has been associated in four patients and neuroretinitis in one. Of the four vascular occlusion cases, one had a macular star. Herein we report an exceptional case of melanocytoma associated with optic nerve head edema, venous stasis retinopathy, and a macular star associated with good visual recovery.

Keywords: tumors • retina • macula/fovea