Pediatric sickle cell retinopathy: A review of screening, diagnostic imaging and treatment

Topic: Sickle cell retinopathy (SCR) Relevance: To synthesize evidence on SCR examining genotype-specific features, multimodal imaging findings, systemic correlations, and management approaches, with the goal of informing earlier and more personalized ophthalmic care for children with sickle cell disease (SCD). Methods: This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A comprehensive PubMed search identified studies published between 1974 and 2025 reporting on SCR in children (≤18 years). Eligible studies included those describing ocular or imaging findings, classification approaches, systemic correlations, or management outcomes in children with SCD. Results: Thirty-seven studies met inclusion criteria. SCR was recognized as an early, often subclinical manifestation of SCD in children, with retinal changes reported as early as 5–7 years of age. Hemoglobin SC genotype was more often associated with proliferative retinopathy, while hemoglobin SS showed earlier macular thinning and anemia-related changes. Despite structural abnormalities, visual acuity generally remained preserved, underscoring the limitations of symptom-based screening. Optical coherence tomography (OCT), OCT angiography (OCTA), and ultra-widefield imaging reveal macular and peripheral pathology not detected on routine examination. Across studies, classification systems were inconsistently applied; most relied on the Goldberg staging designed for adults, highlighting the lack of pediatric-specific criteria. Retinal findings correlated with hematologic markers, hospitalization burden, and cerebrovascular disease, suggesting potential value as systemic biomarkers. Management remains largely observational, with selective use of laser and systemic therapies in advanced cases. Conclusions: Pediatric SCR demonstrates early onset, genotype-dependent variation, and systemic associations. Current evidence supports the role of multimodal imaging for early detection and longitudinal monitoring. Standardizing screening practices and refining existing classification frameworks to reflect pediatric disease patterns may enhance early recognition and long-term visual outcomes. PROSPERO registration number: CRD420251142795.