Retina. 2019 Sep;39(9):1761-1767. doi: 10.1097/IAE.0000000000002222.


Purpose: To assess foveal and parafoveal vasculature at the superficial capillary plexus, deep capillary plexus, and choriocapillaris of patients with X-linked retinoschisis by means of optical coherence tomography angiography.

Methods: Six patients with X-linked retinoschisis (12 eyes) and seven healthy controls (14 eyes) were recruited and underwent complete ophthalmologic examination, including best-corrected visual acuity, dilated fundoscopy, and 3 × 3-mm optical coherence tomography angiography macular scans (DRI OCT Triton; Topcon Corp). After segmentation and quality review, optical coherence tomography angiography slabs were imported into ImageJ 1.50 (NIH; Bethesda) and digitally binarized. Quantification of vessel density was performed after foveal avascular zone area measurement and exclusion. Patients were additionally divided into "responders" and "nonresponders" to dorzolamide therapy.

Results: Foveal avascular zone area resulted markedly enlarged at the deep capillary plexus (P < 0.001), particularly in nonresponders. Moreover, patients disclosed a significant deep capillary plexus rarefaction, when compared with controls (P: 0.04); however, a subanalysis revealed that this damage was limited to the fovea (P: 0.006). Finally, the enlargement of foveal avascular zone area positively correlated with a decline in best-corrected visual acuity (P: 0.01).

Conclusion: Prominent foveal vascular impairment is detectable in the deep capillary plexus of patients with X-linked retinoschisis. Our results correlate with functional outcomes, suggesting a possible vascular role in X-linked retinoschisis clinical manifestations.

Publication types

  • Observational Study

MeSH terms

  • Adolescent
  • Capillaries / diagnostic imaging
  • Capillaries / pathology*
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Fovea Centralis / diagnostic imaging
  • Fovea Centralis / pathology
  • Humans
  • Macular Degeneration / diagnostic imaging
  • Macular Degeneration / pathology
  • Male
  • Microvessels / diagnostic imaging
  • Microvessels / pathology
  • Prospective Studies
  • Retinal Vessels / diagnostic imaging
  • Retinal Vessels / pathology*
  • Retinoschisis / diagnostic imaging
  • Retinoschisis / pathology*
  • Tomography, Optical Coherence