Rétinopathie drépanocytaire : analyse rétrospective portant sur 730 patients suivis dans un centre de référence

Abstract

Sickle-cell disease is the most common genetic disease in the world, frequently complicated by potentially blinding retinal complications. The prevalence of sickle-cell retinopathy in patients followed in a referral center is presented in this study. The prevalence of proliferative sickle-cell retinopathy by Goldberg classification was determined via a retrospective study of primarily adult SS and SC sickle-cell disease patients and AS sickle trait patients followed in a single referral center for a mean period of 13 years. All patients underwent slit lamp examination and complete fundus examination. Seven hundred and thirty patients (mean age 32.5±10 years), consisting of 492 SS patients (67.4%), 229 SC patients (31.4%) and nine AS patients (1.2%), were included in the study. 54.6% of SC patients and 18.1% of SS patients had grade 3 to 5 proliferative sickle-cell retinopathy. The prevalence of severe forms of sickle-cell retinopathy was higher among SS men than among SS women (21.7% versus 15.5%; P<0.05). The high prevalence of sickle-cell retinopathy and the potentially severe complications associated with this disease justify screening and therapeutic management by a multidisciplinary team in the setting of a referral center.