Leber's idiopathic stellate neuroretinitis

Abstract

O ptic disc edema with macular star is a descriptive term encompassing a heterogeneous group of disorders. It is usually idiopathic, but infectious causes, such as syphilis, Lyme disease, cat-scratch disease, toxoplasmosis and viral infection, should be considered. 1-7 In 1916 Leber8 described a syndrome with optic disc edema and macular star. Leber's idiopathic stellate neuroretinitis, originally known as Leber's stellate maculopathy, is a relatively uncommon clinical entity characterized by unilateral optic disc edema followed by the development of a macular star. Exudative deposits in the macula in a stellate pattern are the result of primary optic nerve disease and not retinal inflammation. For this reason some authors have suggested the term idiopathic optic disc edema with a macular star rather than neuroretinitis for idiopathic cases. l The clinical features include severe visual loss, optic disc swelling, exudative detachment of the peripapillary retina, macular star and spontaneous resolution of hard exudates in otherwise healthy young patients.813