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Retinal ultra-wide-field colour imaging versus dilated fundus examination to screen for sickle cell retinopathy
  1. Talal Alabduljalil1,
  2. Crystal SY Cheung2,
  3. Cynthia VandenHoven2,
  4. Leslie D Mackeen2,
  5. Melanie Kirby-Allen2,
  6. Peter J Kertes2,
  7. Wai-Ching Lam2,3
  1. 1 Surgery, Healthy Sciences center, Kuwait University, Jabriya, Kuwait
  2. 2 Ophthalmology and Vision Sciences, University of Toronto, Toronto, Canada
  3. 3 Li Ka Shing Faculty of Medicine, Ophthalmology, University of Hong Kong, Hong Kong, Hong Kong
  1. Correspondence to Talal Alabduljalil Department of Surgery, Faculty of Medicine, Kuwait University, Kuwait; talal.alabduljalil{at}


Purpose To compare ultra-wide-field colour fundus imaging (UWFI) to dilated fundus examination (DFE) for the screening of sickle cell retinopathy (SCR).

Design This study is a prospective, blinded, multicentre case series.

Participants This study included two groups: an adult group (n=268 eyes) and a paediatric group (n=168 eyes). Sickle cell disease (SCD) types included haemoglobin S homozygous (HbSS), haemoglobin S and C (HbSC) and Hb S with β-thalassaemia (HbSß-Thal).

Methods Participants underwent DFE and UWFI. Each eye received three independent grades (1–4), documented by three graders: clinical grader, image grader 1 and image grader 2. Three clinically relevant diagnostic thresholds were determined. Based on these thresholds, the sensitivity, specificity, positive predictive value and negative predictive value for all three graders were calculated relative to each other as reference tests.

Results HbSC was associated with the most advanced SCR grades. When compared to the clinical grader, image grader 1 and image grader 2 consistently detected more SCR and higher SCR grades in both adult and paediatric groups. In both groups, image grader 1 and image grader 2 identified twice as many cases of capillary occlusion/anastomosis than clinical grader. To detect the presence of any proliferative SCR, image grader 1 and image grader 2 had a sensitivity of 82%, 71% in the paediatrics group and 90% and 72% in the adult group. The clinical grader sensitivity was 52% in the paediatrics group and 53% in the adult group.

Conclusion The UWFI is a sensitive tool to screen for SCR. It is superior to DFE in detecting capillary occlusion or anastomosis.

  • Epidemiology
  • Retina
  • Clinical Trial
  • Child health (paediatrics)

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  • Contributors Substantial contributions to the conception or design of the work, or the acquisition, analysis or interpretation of data: TA, W-CL, PK, MK-A. Drafting the work or revising it critically for important intellectual content: TA, CSYC, CVH, LM. Final approval of the version published: TA, W-CL, PK, MK-A, CSYC, CV, LM.

  • Funding This work was supported by the Brandan’s Eye Research Fund (BERF) and Ophthalmology Research Fund (ORF) at SickKids.

  • Competing interests The fundus camera (Optos—Daytona) that was used in the paediatric group of this study was provided on loan from Optos to conduct this research. Authors have no other relevant financial disclosures to this project.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data availability not applicable as no data sets are generated and/or analysed for this study. All data relevant to the study are included in the article or uploaded as supplementary information.

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