June 2015
Volume 56, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2015
Outer segment thickness rather than total retina thickness predicts macular function in X-Linked Retinoschisis (XLRS)
Author Affiliations & Notes
  • Lea D Bennett
    Retina Foundation of the Southwest, Dallas, TX
  • Mark E Pennesi
    Casey Eye Institute, Portland, OR
    Oregon Health and Science Center, Portland, OR
  • Joycelyn Niimi
    Casey Eye Institute, Portland, OR
    Oregon Health and Science Center, Portland, OR
  • David J Wilson
    Casey Eye Institute, Portland, OR
    Oregon Health and Science Center, Portland, OR
  • Laura Erker
    Casey Eye Institute, Portland, OR
    Oregon Health and Science Center, Portland, OR
  • Maria Parker
    Casey Eye Institute, Portland, OR
    Oregon Health and Science Center, Portland, OR
  • John R Heckenlively
    University of Michigan, Ann Arbor, MI
    Kellogg Eye Center, Ann Arbor, MI
  • Kari E Branham
    University of Michigan, Ann Arbor, MI
    Kellogg Eye Center, Ann Arbor, MI
  • David G Birch
    Retina Foundation of the Southwest, Dallas, TX
  • Footnotes
    Commercial Relationships Lea Bennett, None; Mark Pennesi, None; Joycelyn Niimi, None; David Wilson, None; Laura Erker, None; Maria Parker, None; John Heckenlively, None; Kari Branham, None; David Birch, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2015, Vol.56, 3834. doi:
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    • Get Citation

      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.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: 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.

Methods: 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.

Results: 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.

Conclusions: 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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