Retinal Challenges in Sickle Cell Patients-Present and Future: A Systematic Review

Importance: Sickle cell retinopathy and maculopathy are frequent but underdiagnosed complications of sickle cell disease that can lead to vision loss. Unfortunately, there is currently no ophthalmological follow-up protocol to best diagnose and treat these complications. Objective: To identify the most important risk factors associated with the progression of sickle cell retinopathy and maculopathy, review the current screening techniques and effective treatments and propose a follow-up protocol tailored to the patient's risk profile. Evidence Review: Relevant studies were selected using the PICO framework and a structured search strategy across multiple databases including PubMed, Scopus, Cochrane Library, Cible+, ScienceDirect and the American Academy of Ophthalmology network. The search spanned from 1980 to 2025. Original research articles addressing risk factors, screening, and treatment methods for sickle cell retinopathy and maculopathy were included. In total, 48 studies met the inclusion criteria. Data extraction and quality assessment were applied uniformly. Findings: The systematic review included 48 articles (10,543 participants), representing a mix of study types: randomized clinical trials, prospective and retrospective cohort studies, and cross-sectional studies. Major findings included: Risk Factors: Commonly identified risk factors for sickle cell retinopathy and maculopathy include genotype SS, increased age, male sex, and low fetal hemoglobin levels. Screening and Diagnosis: Wide-field fluorescein angiography and optical coherence tomography angiography were the most effective tools for early detection of peripheral ischemia and macular thinning, respectively. Treatment and Management: Laser photocoagulation remains the standard intervention for proliferative sickle cell retinopathy. Preventive strategies and regular ophthalmologic monitoring are emphasized across high-quality studies. Conclusions and Relevance: Risk stratification based on genotype, hemoglobin F levels, and imaging findings allows for tailored ophthalmologic management of patients with sickle cell disease. A structured follow-up protocol is proposed, aiming to improve early detection, prevent complications, and optimize resource allocation.