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Advances in sickle cell retinopathy screening techniques, tests, and practices: A systematic review
Olivia W Cummings1, Sara Rahman2, Lauren Fletcher3
1Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA.
American Journal of Hematology
|July 23, 2024
Summary
Sickle cell retinopathy (SCR) screening can be improved. Advanced imaging like ultra-widefield photography and OCT angiography show superior accuracy for detecting proliferative sickle cell retinopathy (PSR) compared to standard dilated fundus exams.
Area of Science:
- Ophthalmology
- Medical Imaging
- Hematology
Background:
- Sickle cell retinopathy (SCR) is a leading cause of vision loss in sickle cell disease (SCD).
- Current screening relies on dilated fundus examination (DFE), with recommendations based on limited evidence.
- Proliferative sickle cell retinopathy (PSR) signifies advanced disease, potentially leading to blindness.
Conclusions:
- Advanced imaging techniques, including ultra-widefield photography and OCTA, offer superior accuracy for detecting PSR.
- These methods are more effective than DFE for identifying sight-threatening complications of SCR.
- Integration of advanced imaging into SCR screening protocols is supported, pending implementation studies.

