We aimed to describe the epidemiological, etiological and clinical features, treatment and clinical course of sickle cell retinopathy in children and to determine the risk factors for serious involvement.
Methods: This was a retrospective study including all children diagnosed with sickle cell retinopathy. Epidemiological, clinical and therapeutic characteristics, as well as clinical course, were analysed retrospectively by chart review. Two groups were defined: Group 1 (Goldberg stage 1 and 2); Group 2 (Goldberg stage 3, 4 and 5). In order to identify factors independently associated with severe sickle cell retinopathy, we conducted a logistic regression analysis in descending order.
Results: The frequency of sickle cell retinopathy was 14.48%. Forty-two patients (84 eyes) were included; among them 23 boys and 19 girls, aged 10 to 17 with a mean age of 14±1.98 years. Twenty patients were of genotype SS, 11 patients of genotype SC, 8 Sβ and 3 SO Arab. The three patients in group 2 were all of SS genotype. The majority of patients (32) had an HbF level of less than 15%. All our patients had sickle cell retinopathy distributed as follows: 62% at stage 1; 31% at stage 2; 5% at stage 3 and 2% at stage 4. Multivariate analysis revealed a single risk factor independently linked to severe involvement - an HbF level<15%.
Conclusion: Retinopathy is a frequent complication of sickle cell disease which may lead to blindness. The HbF level is negatively correlated with severe involvement.
Keywords: Drépanocytose; Genotype; Génotype; Neovascularisation; Néovascularisation; Retinopathy; Rétinopathie; Sickle cell disease.
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