Analysis of anatomic and functional measures in X-linked retinoschisis

Catherine A. Cukras, Laryssa A. Huryn, Brett P. Jeffrey, Amy Turriff, Paul A. Sieving

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


PURPOSE. To examine the symmetry of structural and functional parameters between eyes in patients with X-linked retinoschisis (XLRS), as well as changes in visual acuity and electrophysiology over time. METHODS. This is a single-center observational study of 120 males with XLRS who were evaluated at the National Eye Institute. Examinations included best-corrected visual acuity for all participants, as well as ERG recording and optical coherence tomography (OCT) on a subset of participants. Statistical analyses were performed using nonparametric Spearman correlations and linear regression. RESULTS. Our analyses demonstrated a statistically significant correlation of structural and functional measures between the two eyes of XLRS patients for all parameters. OCT central macular thickness (n = 78; Spearman r = 0.83, P < 0.0001) and ERG b/a ratio (n = 78; Spearman r = 0.82, P < 0.0001) were the most strongly correlated between a participant’s eyes, whereas visual acuity was less strongly correlated (n = 120; Spearman r = 0.47, P < 0.0001). Stability of visual acuity was observed with an average change of less than one letter (n = 74; OD -0.66 and -OS 0.70 letters) in a mean follow-up time of 6.8 years. There was no statistically significant change in the ERG b/a ratio within eyes over time. CONCLUSIONS. Although a broad spectrum of clinical phenotypes is observed across individuals with XLRS, our study demonstrates a significant correlation of structural and functional findings between the two eyes and stability of measures of acuity and ERG parameters over time. These results highlight the utility of the fellow eye as a useful reference for monocular interventional trials.

Original languageEnglish (US)
Pages (from-to)2841-2847
Number of pages7
JournalInvestigative Ophthalmology and Visual Science
Issue number7
StatePublished - Jun 2018
Externally publishedYes


  • Longitudinal
  • Natural history
  • X-linked retinoschisis

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience


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