Sickle cell disease retinopathy: characterization among pediatric and teenage patients from northeastern Brazil

OBJECTIVE The aim of the present study was to characterize sickle cell disease retinopathy in children and teenagers from Bahia, the state in northeastern Brazil with the highest incidence and prevalence of sickle cell disease. METHODS A group of 51 sickle cell disease patients (36 hemoglobin SS and 15 hemoglobin SC) with ages ranging from 4 to 18 years was studied. Ophthalmological examinations were performed in all patients. Moreover, a fluorescein angiography was also performed in over 10-year-old patients. RESULTS The most common ocular lesions were vascular tortuosity, which was found in nine (25%) hemoglobin SS patients, and black sunburst, in three (20%) hemoglobin SC patients. Peripheral arterial closure was observed in five (13.9%) hemoglobin SS patients and in three (13.3%) hemoglobin SC patients. Arteriovenous anastomoses were present in six (16.5%) hemoglobin SS patients and six (37.5%) hemoglobin SC patients. Neovascularization was not identified in any of the patients. CONCLUSIONS This study supports the use of early ophthalmological examinations in young sickle cell disease patients to prevent the progression of retinopathy to severe disease and further blindness.