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Han, Ian C., MD*,†; Zhang, Alice Y., MD*; Liu, Tin Y. A., MD*; Linz, Marguerite O., BA*; Scott, Adrienne W., MD*

doi: 10.1097/IAE.0000000000002057
Original Study

Purpose: To determine whether ultra-widefield (UWF) retinal imaging changes the staging or management of sickle cell retinopathy compared with clinical examination.

Methods: Prospective, observational study including patients with sickle cell disease. All patients underwent dilated fundus examination by a fellowship-trained retina specialist, as well as UWF fundus photography (FF) and fluorescein angiography (FA). Sickle retinopathy stage and treatment recommendation per eye were determined after clinical examination, UWF-FF, and UWF-FA, respectively, and differences in retinopathy stage and treatment recommendation were compared.

Results: A total of 70 eyes from 35 patients (17 women, 48.6%), mean age 30.4 years, were included. Sickle genotypes included 26 patients with sickle SS (74.3%), 7 SC (20.0%), and 2 β(+)thalassemia (5.7%). Based on examination, most eyes (42/70; 60.0%) had no visible retinopathy. Based on UWF-FF, about half of the eyes were found to be Goldberg Stage 2 or above (36/70; 51.4%). Based on UWF-FA, nearly all eyes were Goldberg Stage 2 or above (63/70; 90%). However, clinical examination reliably detected neovascularization, and in no case did the addition of UWF imaging change management relative to examination alone.

Conclusion: Ultra-widefield imaging detects a higher stage of sickle cell retinopathy compared with clinical examination alone, but these differences may not be clinically significant.

Ultra-widefield retinal imaging is useful for the diagnosis and staging of sickle cell retinopathy. Ultra-widefield imaging detects a higher stage of sickle retinopathy compared with clinical examination alone, but the greater detection of peripheral pathologic changes did not alter treatment decisions in this study.

*Retina Division, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland; and

Department of Ophthalmology and Visual Sciences, Carver College of Medicine, University of Iowa, Iowa City, Iowa.

Reprint requests: Adrienne W. Scott, MD, Retina Division, Wilmer Eye Institute, 600 North Wolfe Street, Maumenee 719, Baltimore, MD 21287; e-mail:

Supported through unrestricted contributions to the Johns Hopkins University Retina Division research fund, and in part by private philanthropy from Gail C. and Howard Woolley.

Presented in part at the 49th Annual Meeting of the Retina Society, San Diego, CA, September 15, 2016.

None of the authors has any financial/conflicting interests to disclose.

© 2019 by Ophthalmic Communications Society, Inc.