TEMPORAL THINNING IN SICKLE CELL RETINOPATHY IS ASSOCIATED WITH DIMINISHED PERFUSION ON OCTA AND DENSE SCOTOMA ON MICROPERIMETERY.

PURPOSE

To establish correlation between the structural compromise (capillary loss and hypoperfusion on optical coherence tomography [optical coherence tomography angiography]) with perimacular functional impairment noted on microperimetry.

METHODS

Retrospective case series. Clinical records and multimodal imaging findings of patients presenting with sickle cell disease were reviewed.

RESULTS

Three eyes of three patients (two men) were included in the study with a visual acuity of 20/20 and no sickle cell retinopathy. Images using optical coherence tomography angiography (AngioVue OCT angiography system; Optovue, Inc, Fremont, CA) were obtained along with spectral domain optical coherence tomography, fluorescein angiography, and microperimetry (MP-1). Spectral domain optical coherence tomography revealed selective loss of inner retinal layers with thinning of the retina. Optical coherence tomography angiography revealed compromise of both superficial and deep capillaries in the area of temporal thinning expressed on spectral domain optical coherence tomography. MP-1 demonstrated focal increase in threshold (decreased sensitivity) correlating with the perfusion defects on optical coherence tomography angiography. Fluorescein angiography did not show any substantial perfusion compromise.

CONCLUSION

Optical coherence tomography angiography may reflect the extent of functional compromise even before it being evident on fluorescein angiography. The area vascular compromise was larger in the deep plexus compared with the superficial plexus. The area of complete loss of retinal sensitivity corresponds to loss of vasculature in both the superficial and deep plexuses, whereas the area of decreased sensitivities corresponds to compromise only in the deep plexus.