Sickle cell retinopathy From pathogenesis to management

Sickle cell disease is a hereditary hemoglobinopathy characterized by abnormal red blood cell deformability and vaso-occlusion, which can lead to multi-organ complications, including the eye. Sickle cell retinopathy and sickle cell maculopathy are important ocular manifestations, ranging from peripheral ischemia and neovascularization to macular microvascular damage, potentially resulting in visual impairment. This thesis advances our understanding of these retinal conditions, with a focus on their pathogenesis, natural history, progression, and clinical management. The findings demonstrate that retinal complications in sickle cell disease arise from complex and multifactorial mechanisms, involving interactions between hematological abnormalities and microvascular dysfunction.
Through clinical studies, advanced imaging, and longitudinal follow-up, this work provides new insights into disease progression and the clinical relevance of subclinical abnormalities. Despite these advances, important knowledge gaps remain, particularly regarding pathophysiological mechanisms and predictors of disease progression. As life expectancy in patients with sickle cell disease continues to increase, optimizing ophthalmic care becomes increasingly important. This thesis highlights the need to improve screening strategies and develop more personalized management approaches. Overall, this work provides a strong foundation for future research and underscores the importance of close collaboration between ophthalmologists and hematologists to improve the detection, monitoring, and management of ocular complications in sickle cell disease.