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F. Lalloum, N. Leveziel, V. Letien, M. Binaghi, F. Galacteros, E. H. Souied; Sickle Cell Disease Retinopathy : A Retrospective Study of 730 Patients. Invest. Ophthalmol. Vis. Sci. 2010;51(13):2602.
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Sickle cell disease (SCD) is the most common genetic disease, with a prevalence of 2.3% in the world (120 million) carrying a mutation. The retinopathy associated to sickle cell disease is potentially severe. The purpose of this study is to assess the prevalence of different stages of retinopathy associated to SCD.
Monocentric retrospective study on patients followed in the Adult Sickle-Cell Referral Center at our university hospital. The clinical examination included measurement of visual acuity, slit lamp examination, fundus examination and fluorescein angiography.
730 patients with SCD (32.5 ± 10.07 years) have been studied. 492 homozygous SS patients (103 men, 289 women, mean age 35.7 ± 10.2 years), 229 SC heterozygous patients (98 men, 131 women, 44,4 ± 10.4 years) and 9 AS patients (4 men, 5 women, 37 ± 14.2 years) were included in this study. Among the homozygous patients, 318/492 (64.63%) had peripheral ischemia, 87/492 (17.68%) had peripheral active neovascularisation or fibrosis, and 6/492 had unilateral blindness (1, 21%). Among heterozygous patients, 184/229 (80%) had peripheral retinal ischemia, 125/229 (54.34%) had peripheral neovascularisation or fibrosis, and 13/229 (5,65%) had unilateral blindness. The peripheral néovascularisation and unilateral blindness were more prevalent in the heterozygous SC patients than in homozygous patients (p<0.05). Among AS patients, 2/9 (22.22%) had retinal ischemia, none exhibited neovascularization or blindness.
Sickle cell retinopathy is a common complication of the disease. The risk of visual loss is 4.7 times more frequent in heterozygous SC than in homozygous SS patients. Laser photocoagulation is important to prevent the development of retinal complications related to neovascularization.
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