Surgical management of sickle cell retinopathy: a systematic review of clinical outcomes and a single-arm meta-analysis of single-surgery anatomical success.

Abstract

Introduction: Sickle cell retinopathy is a consequence of sickle cell disease that results from vascular complications. Sickle cell disease is predominantly prevalent in Africa, evidenced by 20% to 30% of the population in Gabon, Cameroon, and Ghana being affected by it. This systematic review aims to evaluate the surgical outcomes of patients with sickle cell retinopathy to inform evidence-based management of late stages of sickle cell retinopathy.

Methods: This systematic review was conducted in accordance with the PRISMA 2020 guidelines. A Literature search was conducted on PubMed, Embase (Ovid), and Cochrane (CENTRAL) on 05/08/2025. Inclusion criteria included studies with patients with sickle cell retinopathy who underwent surgery and reported anatomical and/or visual outcomes. A random effects single-arm meta-analysis was carried out in the anatomical success group using JASP software, and the risk of bias was evaluated using JBI and RoB2 tools.

Results: 7 studies, comprising 408 eyes, were included. Overall, the evidence showed that surgical treatment in patients with sickle cell retinopathy results in improved final visual acuity and high single-surgery anatomical success (SSAS, between 60% to 83%). The pooled SSAS data, based on logit-transformed proportions, yielded an effect of 0.83 (95% CI: 0.24-1.42), corresponding to a pooled SSAS of approximately 70% (95% CI: 56-80%), with low, non-significant heterogeneity among the included studies (Q (4) = 5.61, p = 0.230; I² = 29.73% [0.00, 92.46]). Leave-one-out sensitivity analysis confirmed that the estimate was robust, with the pooled SSAS ranging from approximately 66% to 74% across iterations.

Discussion: Studies have reported a considerable gain in final visual acuity and anatomical success in patients undergoing surgery for sickle cell retinopathy. The small number of studies and small sample sizes increase heterogeneity. Therefore, although the results indicate favourable surgical outcomes, clinicians must weigh up the benefits of surgery against possible complications. In conclusion, more studies are warranted to guide evidence-based medicine.

Systematic review registration: https://www.crd.york.ac.uk/PROSPERO/view/CRD420251165357, identifier CRD420251165357.