Abstract

Purpose: This article aims to describe the prevalence of retinal alterations on the indirect binocular ophthalmoscopy exam in patients with SS and SC Sickle disease who are younger than 10 years of age in the city of Salvador in Brazil.

Methods: This is a retrospective study in which patients with sickle cell disease with an age group of 10 years or younger were attended in a service of retina in Salvador, Brazil in the last 10 years. All patients were submitted to the clinical file filling, which includes the sociodemographic profile, clinical profile and ophthalmologic examination. The patients were divided into two groups (SS or SC), according to the genotypic profile of hemoglobinopathy. The classification of retinopathy was performed according to Goldberg in proliferative and non-proliferative retinopathy. A P-value < 0.05 was considered statistically significant.

Results: A total of 90 patients (180 eyes) were evaluated, which 65 (72%) had SS sickle cell anemia and 25 (28%) had SC sickle disease. Of the 90 patients, 56 (62%) did not present retinal changes and 34 (38%) present sickle retinopathy. Of the 34 patients with retinopathy, 12 (35%) had non-proliferative sickle retinopathy and 22 (65%) had proliferative alterations. The increase in vascular tortuosity was the most observed non-proliferative sign (15,5% of eyes). It was observed that the two groups presented a similar proportion of areas of retinal non-perfusion (p = 1).

Conclusion: The results suggest the need for regular ophthalmologic follow-up of patients with sickle cell disease, even younger than 10 years of age, since in this study findings of non-proliferative sickle retinopathy were found in patients from 1 year of age and proliferative disease from 5 years.

Keywords: Sickle Cell Anemia; Hemoglobin SC Disease; Retina; Retinal Diseases; Epidemiology

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