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Lea D Bennett, Mark E Pennesi, Joycelyn Niimi, David J Wilson, Laura Erker, Maria Parker, John R Heckenlively, Kari E Branham, David G Birch; Outer segment thickness rather than total retina thickness predicts macular function in X-Linked Retinoschisis (XLRS). Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):3834. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
An ongoing, multi-center, prospective natural history study of XLRS was designed to understand disease progression and determine suitable outcome measures for upcoming gene therapy trials. Here we assess baseline data in order to evaluate the relationship between spectral domain optical coherence tomography (sdOCT) measures and psychophysical measures of macular function in patients with XLRS.
Male subjects with a documented mutation in the gene Retinoschisin (RS1) were evaluated at a baseline visit. Outcome measures were best-corrected visual acuity (BCVA), sdOCT, full-field electroretinography (ffERG), and microperimetry. The average thickness of the outer segments (OS) was measured by segmentation (IGOR) at the central 100. Data from microperimetry was collected using the 4-2 threshold testing strategy (spot size 3, 68 points). Sensitivity was measured over 100 (6 points separated by 2° each) of the horizontal midline corresponding to the sdOCT scan. ffERGs were performed according to ISCEV standards. Correlation was determined with Pearson’s r correlation.
To date, 15 male subjects (13- 66 years-of-age) with average BCVA of 0.4 ± 0.05 logMAR have been evaluated. Patients had negative ffERG responses typical of XLRS. The average thickness of OS was 45.4 ± 29.9 % below mean age-matched normal whereas the average full retinal thickness was 13.9 ± 45.2 % thicker than normal. The corresponding retinal sensitivity was decreased by 40.4 ± 27.0 %. Photoreceptor OS thickness had a strong association with BCVA (r = -0.593; p = 0.015) and a very strong correlation with retinal sensitivity (r = 0.792; p < 0.001). However there was no association between full retinal thickness and either acuity or macular sensitivity.
When considered from a single visit, the OS thickness is better at predicting macular function and BCVA than total retinal thickness in patients with XLRS.
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