Foveal avascular zone morphology and parafoveal capillary perfusion in sickle cell retinopathy

Br J Ophthalmol. 2020 Apr;104(4):473-479. doi: 10.1136/bjophthalmol-2019-314567. Epub 2019 Jul 23.

Abstract

Background/aims: To assess foveal avascular zone (FAZ) morphology and parafoveal capillary perfusion in patients with various stages of sickle cell retinopathy (SCR) using optical coherence tomography angiography (OCT-A).

Methods: This is a multi-institutional retrospective study of patients with various stages of SCR compared with healthy controls. Parafoveal OCT-A images obtained using a commercial spectral domain-OCT system were reviewed. Foveal-centred 3×3 mm full vascular slab OCT-As were used for image processing and data analysis. FAZ area, perimeter, and acircularity index were determined on the OCT-A image after manual delineation of the FAZ border. Quadrant-based parafoveal capillary density and per cent area deviating from normal distribution were also measured.

Results: Fifty-two patients with SCR (33 non-proliferative and 19 proliferative) and 20 age and race-matched healthy controls were included. One randomly selected eye per study participant was analysed. FAZ perimeter and acircularity index were significantly greater in SCR eyes when compared with the controls. While parafoveal capillary density was significantly lower, per cent area deviated from normal distribution was significantly higher in SCR eyes than that of the control. However, no statistically significant difference between the two SCR stages was observed. In quadrant-based analysis, the temporal quadrant showed greater parafoveal capillary dropout due to SCR, with the most profound effect in patients with proliferative SCR.

Conclusions: Abnormal FAZ morphology and altered parafoveal capillary perfusion were found in patients with SCR. Our customised OCT-A image analysis method uniquely highlights significant quantitative alterations in perfusion density mapping in a qualitative display, with minimal obscuration of OCT-A image detail.

Keywords: imaging; macula; retina.

Publication types

  • Comparative Study
  • Multicenter Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Anemia, Sickle Cell / diagnostic imaging
  • Anemia, Sickle Cell / physiopathology*
  • Capillaries / diagnostic imaging
  • Capillaries / physiopathology*
  • Female
  • Fluorescein Angiography
  • Fovea Centralis / blood supply*
  • Humans
  • Ischemia / diagnostic imaging
  • Ischemia / pathology*
  • Male
  • Middle Aged
  • Retinal Diseases / diagnostic imaging
  • Retinal Diseases / physiopathology*
  • Retinal Vessels / diagnostic imaging
  • Retinal Vessels / pathology*
  • Retrospective Studies
  • Tomography, Optical Coherence
  • Visual Acuity
  • Young Adult