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Tomas R. Burke, David W. Rhee, Stephen H. Tsang, Theodore Smith, Rando Allikmets, Margaret A. Lazow, Stanley Chang, Donald C. Hood, Vivienne C. Greenstein; Peripapillary Sparing And Macular Involvement In Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2011;52(14):5010.
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To compare structure and function of the macula and peripapillary retina in Stargardt disease to see if there is relative sparing of the peripapillary area.
Sixteen eyes of 16 patients with Stargardt disease and 12 eyes of 12 age-similar controls were studied. The ABCR 500 micro-array was used for genotyping. Patients were classified based on full-field electroretinogram (ERG) results. Fundus autofluorescence (FAF) and spectral domain-optical coherence tomography (SD-OCT) (Heidelberg Spectralis HRA+OCT) horizontal line scans through the foveal and peripapillary areas were obtained. The thicknesses of the outer nuclear layer + outer plexiform layer (ONL+), outer segment (OS) and retinal pigment epithelium (RPE) through the foveal area and the peripapillary area from 1° to 4° temporal to the optic disc were measured using a computer aided manual segmentation technique. Visual sensitivity in the central 10° and the peripapillary area was assessed with the Nidek MP-1 microperimeter. Total deviation plots were generated. Visual field sensitivities were compared to thicknesses of the underlying retinal layers.
All patients had at least 1 mutation detected in the ABCA4 gene. Thirteen patients had ERG group I and 3 had group II disease. For all patients SD-OCT line scans showed significant thinning of the ONL+ and significant thinning or absence of the OS layers. The ONL+ layer was significantly thinner even in regions with preserved inner segment/OS junctions. The RPE was significantly thinner in 10 patients. A greater correlation of visual sensitivity with OS (r2:0.66) compared to ONL+ thickness (r2:0.45) was demonstrated. At eccentricities >8° from the fovea the ONL+, OS and RPE thickness values were less affected in 10, 5 and 9 patients, respectively, at locations in the nasal compared with temporal retina. Of the 9 patients where the peripapillary area was studied from 1° to 4° from the optic disc, there was only 1 patient, with ERG group II disease, who showed significant thinning of the ONL+ and absence of the OS layer. This patient had abnormal peripapillary FAF.
The SD-OCT and MP-1 results, consistent with previous reports using en-face imaging, showed relative sparing of the peripapillary area in Stargardt disease and provide quantitative markers of disease. 1. Lois et al.(2001) Arch Ophthalmol;119:359-69. 2. Hood et al.(2009) IOVS;50:2328-36.
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