Retinal angiography and optical coherence tomography disclose focal optic disc vascular leakage and lipid-rich fluid accumulation within the retina in a patient with leber idiopathic stellate neuroretinitis.

A 52-year-old woman with clinical features of Leber idiopathic stellate neuroretinitis (LISN) underwent retinal fluorescein and indocyanine green angiography that revealed lipid-containing fluid leakage from a single arteriole in the superficial nerve fiber layer of the optic disc. The fluid expanded gradually into the upper half of the optic disc and the adjacent peripapillary retina. Optical coherence tomography (OCT) demonstrated fluid accumulation in two separate subretinal spaces and in the outer nuclear-plexiform layer, which extended from the optic disc margin to the fovea. These angiographic and OCT findings support the hypothesis that LISN develops from focally increased permeability of an optic disc surface arteriole from which lipid-rich fluid flows through the outer nuclear-plexiform layer space to pool in these retinal areas.