Background: Melanocytomas of the optic disk are heavily pigmented benign tumors that are most often discovered during routine examination, as they are classically asymptomatic. Although a slight impairment of visual acuity attributable to the tumor may occur in large tumors, acute profound visual loss is extremely unusual.
Methods: A case involving a 24-year-old woman with melanocytoma of the optic nervehead in the right eye, who experienced acute severe visual loss, an afferent pupillary defect, and optic nervehead edema, is discussed. Laboratory tests and serology for Toxoplasmosis, Syphilis, Borrelia, and Leptospira were performed.
Results: The severe loss of vision, optic disc swelling, exudative detachment of the peripapillary retina, macular star, and spontaneous resolution in an otherwise healthy patient were all consistent with Leber's stellate idiopathic neuroretinitis. Results of serologic testing were not contributory.
Conclusion: This is the first report of Leber's neuroretinitis associated with melanocytoma of the optic nervehead, and should be included in the differential diagnosis of neuroretinitis. The possible mechanisms responsible for this association are discussed.