THREE-DIMENSIONAL CHARACTERISTICS OF FOUR MACULAR INTRARETINAL LAYER THICKNESSES IN SYMPTOMATIC AND ASYMPTOMATIC CARRIERS OF G11778A MUTATION WITH LEBER'S HEREDITARY OPTIC NEUROPATHY.

Abstract

To characterize by spectral domain optical coherence tomography the three-dimensional thicknesses of four macular intraretinal layers in symptomatic and asymptomatic carriers of G11778A mutation with Leber's hereditary optic neuropathy. Twenty-five eyes (7 symptomatic eyes and 18 asymptomatic eyes) of patients with Leber's hereditary optic neuropathy from one Chinese family and 16 normal eyes were enrolled. Macular radial scans by spectral domain optical coherence tomography and custom software produced intraretinal three-dimensional thickness maps. The macula was divided into nine regions, and each region included four intraretinal layers: nerve fiber layer, ganglion cell layer and inner plexiform layer, inner nuclear layer and outer plexiform layer, and the outer retinal layer. Nerve fiber layer in the symptomatic eyes was significantly thinner than in normal eyes for most of the macular regions; however in the asymptomatic eyes, it was increased in three regions. Ganglion cell layer and inner plexiform layers in all regions of symptomatic eyes were significant thinner than in asymptomatic eyes and controls. Inner nuclear layer and outer plexiform layers in six regions of symptomatic and asymptomatic eyes were significantly thicker than in controls. The outer retinal layer of asymptomatic eyes was thicker than in most control regions. Intraretinal thickness changes in asymptomatic patients could be prodromal events that indicate the imminent conversion to symptomatic patients with Leber's hereditary optic neuropathy.