[Screening for sickle cell disease retinopathy in the north of France]. / Rétinopathie drépanocytaire chez les adultes de la région Nord-Pas-de-Calais.

OBJECTIVE: To study the frequency and the severity of retinopathy in adult patients with sickle cell disease in the north of France. PATIENTS AND METHODS: Patients with sickle cell disease aged more than 18 years old were consecutively referred for screening of sickle cell retinopathy in the ophthalmology department from December 2005 to March 2008. Complete ophthalmologic examination with dilated funduscopy was performed in each patient. Fluorescein angiography was performed in cases with ischemic retinopathy. Goldberg and Penman classifications were used to grade retinopathy severity. RESULTS: Fifty-five patients (100 eyes) were included (26 S/S, 9 S/C, 12 S/B, 3 S/S with hereditary persistence of fetal hemoglobin). Sickle cell retinopathy is more severe in patients with the S/C genotype than the S/S and S/B genotypes. Proliferative retinopathy is more common in S/C patients than S/S and S/B patients (S/C vs S/S, p=0.001; S/C vs SB, p=0.01). A qualitatively abnormal vascular border (type II) is also more frequent in S/C patients than S/S and S/B patients (S/C vs S/S, p=0.001; S/C vs SB, p=0.01). No proliferative retinopathy was found in patients with S/S and hereditary persistence of fetal hemoglobin syndrome. CONCLUSIONS: Screening is useful to detect sickle cell retinopathy, particularly in the adult population, to prevent complications from proliferative retinopathy.