Abstract
Purpose :
The role of regular ophthalmic screening in patients with sickle cell retinopathy is controversial and there are no studies guiding the frequency of review,hence the scope of this study.
Methods :
Retrospective observational study of 66 eyes of 33 patients (20 females, median age 30 years, age range 17-60 years) with sickle cell disease screened for retinopathy with fundoscopy,ultrawide-field pseudocolour fundus pictures (Optos plc, Dunfermline, UK) and OCT-angiography. Type and progression of retinal findings were recorded at each visit.
Results :
25 patients had SS (sickle cell anemia) and 8 patients had SC (sickle cell/hemoglobin C compound). Ethnicity was African in 21 patients, Caribbean in 8 patients, Asian in 2 patients and Caucasian in 2 patients. 9 patients were on systemic treatment (8 hydroxycarbamide, 1 blood transfusions), 24 were not on treatment (1 received allogenic transplant), 6 of whom refused treatment. Median follow-up duration was 18 months (range 0-47 months) and median number of visits was 2. Retinopathy was observed in 39 eyes (unilateral in 7 patients and bilateral in 16 patients). Retinal abnormalities included peripheral vascular closure (24 eyes, 15 patients), black sunbursts (17 eyes, 13 patients), sea-fan neovascularization (9 eyes, 5 patients) (Figure 1) and pale retinal patches (5 eyes, 3 patients). At baseline, there was no statistically significative difference in the distribution of the retinal abnormalities according to disease subtype (p>0.05) and according to treatment group. Over the follow-up period, retinopathy progression was observed in 4 eyes from 2 patients (6,3%) affected by SS who refused systemic treatment. Progression consisted in new retinal hemorrhages (1 eye), new sea-fans (1 eye) or fibrosis enlargement (2 eyes), and was not vision threatening. In 4 eyes from 2 SS patients the OCT-A showed foveal avascular zone enlargement and superficial and deep capillary plexus drop-out (Figure 2).
Conclusions :
No correlation was found between disease subtype and retinal abnormalities. Progression of retinopathy was detected in a small number of patients refusing systemic treatment that should be considered.Given the low rate of progression, a low frequency in monitoring sickle cell retinopathy would be adequate. Studies on larger cohort of patients are needed to confirm our clinical impression.
This is a 2021 ARVO Annual Meeting abstract.