Purpose: To identify retinal manifestations of sickle cell hemoglobinopathy and compare their prevalences among different hemoglobinopathy genotypes in a population of a universitary hospital in Porto Alegre - Brazil. Methods: Ophthalmologic evaluation with fundoscopic examined and genotypic study were performed in sickle cell hemoglobinopathy patients. Results: 94 eyes from 47 patients with sickle cell hemoglobi-nopathy were examinated - 17 men and 30 women, aged 22.4 ± 16.8 (SD) years. 20 patients were found to have SS genotype, 10 SC, 10 AS and 7 SThal. The retinal abnorma-lities seen were salmon patch hemorrhage in 1 eye, black sunburst hyperpigmentation in 12 eyes, sea fan neovas-cularization in 3 eyes, vitreous haemorrhage in 1 eye and retinal detachment in 1 eye. Sixty percent of patients in the SC group presented fundoscopic alterations, showing a 4.0 times greater relative risk for retinopathy when com-pared with the homozygous group (SS) (p<0.05). All 5 eyes with proliferative disease had SC genotype. Conclusions: The frequency as well as the severity of retinal manifestations of sickle cell hemoglobinopathy are higher in the SC heterozigous group when compared with other genotypes.
Sickle cell disease; Sickle cell retinopathy
