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Fundoscopic findings in children with sickle cell hemoglobinopathy in Rio Grande do Norte state

Purpose: To point out the main fundoscopic findings in children with sickle cell trait hemoglobinopathies. Methods: We studied 26 patients with sickle cell hemoglobinopathies in the ophthalmological ward at the Hospital Universitário Onofre Lopes, Natal, RN. The patients were submitted to a research questionnaire and the results were statistically evaluated by the chi² test. Results: The average age was 10,6 years, with visual acuity equal or better than 20/25 in the majority, except for three eyes which presented other associated pathologies. The most frequent type was SS in 57.7% (15/26) of cases, followed by SC e SA in 15.4% (4/26) each, and by S-Thal in 11.5% (3/26). The frequency of sickle cell retinopathy was higher after 10 years of age, being more frequent, in relative values, in type S-Thal (100%) and, in absolute values, in type SS (9 cases). The two most common findings were venous tortuosity (12/26) and "black sunburst" (7/26). Conclusion: We observed that the incidence of retinopathy in sickle cell increased with age over 10 years and no evidence of proliferative disease was found. Therefore, we emphasize the need for early ophthalmological examination in carriers of sickle cell disease as a form of preventing future complications.

Sickle cell disease; Hemoglobinopathies; Fundus oculi; Hemoglobin C disease; Hemoglobin SC disease; Retinal diseases; Children


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