Abstract
Purpose :
To identify individuals with sickle cell retinopathy who are at risk for proliferative disease and to describe the clinical outcome of patients with proliferative sickle cell retinopathy (PSCR).
Methods :
A retrospective chart review was conducted for patients with sickle cell disease (SCD) who underwent an eye examination at Howard University Hospital between August 2016 and March 2022. Age, sex, sickle cell disease genotype, sickle cell retinopathy severity, refractive error, and visual acuity at baseline and final recorded visit were reviewed. Additionally, the utilization of vitrectomy, laser, and hydroxyurea therapy as treatment methods were documented. The Snellen measurements of visual acuity were converted to logMAR for comparison. We categorized vision loss in PSCR patients as follows: < 20/70 (impaired), < 20/200 (legal blindness), and < 20/800 (severe vision loss) in at least one eye, and then compared baseline to final vision. Multivariate regression was used for analysis.
Results :
This study included 202 patients (mean age, 35.8 + 16.1 years; 60% male). Eighty-five (42%) patients demonstrated sickle cell retinopathy, 28 (33%) with non-proliferative retinopathy and 57 (67%) with PSCR. Patients with PSCR had the following genotypes: 12 (21%) SS, 37 (65%) SC, 4 (7%) thalassemia, and 4 (7%) sickle cell trait. Multivariate regression identified age > 35 years old (OR 2.86; P=0.006; 95% CI, 1.35 - 6.06) and SC disease (OR 4.79; P= 0.00; 95% CI, 2.06 -11.12) as risk factors for PSCR. The percentage of patients with PSCR that developed a vision-threatening complication was 61%. Vision loss in patients with PSCR was: <20/70 (46%), <20/200 (31%), and 20/800 (23%). Laser and/or vitrectomy were performed in 34 (60%) patients.
Conclusions :
In this study, a mean age >35 and SC genotype were predictors of PSCR. Complications from PSCR represent a significant cause of vision loss among patients with SCD. Further studies are needed on how to identify the best methods for early detection and optimal treatment of patients with PSCR.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.