RESUMO
The purpose of this prospective study was to evaluate retinal damage in patients with sickle cell disease and its links with the different genotypic forms of the disease in patients consulting at the African Tropical Ophthalmology Institute (IOTA). A total of 38 patients with the HbS gene were included over a 12-month study period. Retinal damage was assessed by a computerised angiofluorography in 31 patients. Of the 38 patients studied, 71% had sickle cell disease (SC), 21% had sickle cell trait (AS) and 8% had sickle cell anemia (SS). Sixty-eight percent of patients (n = 21) presented sickle cell retinopathy. The age group with the highest prevalence of proliferative neovascularisation was between 26 and 35 years. Retinopathy was more frequent in SC patients than AS patients: 90% (n = 19) versus 10% (n = 2). None of the 3 SS patients presented retinopathy. Retinal neovascularisation was the most common finding in the 27 affected eyes. This study confirms the frequency and severity of retinal damage in patients with the HbS haemoglobin, particularly among young people with double heterozygous disease (SC) in the tropical African environment. Treatment of this disorder is largely unavailable to patients in sub-Saharan Africa except at the major eye care centres. An early screening and management programme for retinal damage related to SC would reduce ocular complications and optimise visual efficiency in these young active patients.