Visual outcome of treating proliferative sickle cell retinopathy in 108 eyes

Aim: To report treatment methods and visual outcome of treating proliferative sickle cell retinopathy (PSCR).

Design: Retrospective interventional.

Methods: Review of PSCR eyes treated between 2017 to 2022. Patient demographics, fundus findings at presentation, genotype, PSCR stage, treatment used, and visual outcome were assessed.

Results: 108 eyes of 88 consecutive patients were studied. Male: Female 48:40. Mean age: 38.91 (SD:12.52) years. Genotype: sickle cell haemoglobin C (SC) 83 eyes (76.9%), sickle cell haemoglobin S (SS) 19 eyes (17.6%), and sickle cell trait (AS) 6 eyes (5.5%). PSCR stages: 3: 15 eyes (11.0%), 4: 74 eyes (67.0%), and 5: 19 eyes (22.0%). Treatment methods: Intravitreal Injection (IVI) of anti-vascular endothelial growth factor (VEGF) only (27 eyes,25%), scatter retinal laser photocoagulation (SRLP) only (7 eyes, 6.5%), Vitrectomy + SRLP (29 eyes, 26.9%), IVI + SRLP (25 eyes, 23.1%), and Vitrectomy + IVI + SRLP (20 eyes, 18.5%). The treatment used correlated with PSCR stage (p = 0.000). IVI only was mostly used to treat stage 4 (81.4%), and SRLP only was used for stages 3 (42.9%) and 5 (57.1%). IVI + SRLP treated eyes had the best pre- and post-treatment vision. Vitrectomy + SRLP treated eyes had the most improved vision. SRLP only had least visual improvement. Fundus findings correlated with visual outcome (p = 0.003); but stage of PSCR, genotype and treatment used had no correlation (P > 0.05).

Conclusion: Several options effectively treat PSCR. Visual outcome improved or remained same in 90.7% of treated eyes. Randomized controlled trials will determine the optimum treatment for each distinct presentation of PSCR. Treatment guidelines and a disease classification system of prognostic value are unmet needs.