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Retinal impairment in young individuals with sickle cell anemia (hemoglobin SS disease) in university hospital in Northeastern of Brazil

PURPOSES: To describe and categorize retinal vascular changes in patients with sickle cell anemia, as well as to compare diagnostic methods (indirect ophthalmoscopy and fluorescein angiography). METHODS: Patients with sickle cell anemia over the age of seven were examined. Complete ophthalmologic examination with indirect ophthalmoscopy and angiography was performed in each patient. The fundoscopy results were grouped in 3 classes: normal; non-proliferative retinopathy, which includes vascular tortuosity, black sunburst, salmon-patch and peripheral closure/anastomoses; and proliferative retinopathy, related to neovascular proliferation. Angiography results were classified according to Goldberg classifications from stage I to V. RESULTS: Retinopathy related to sickle cell anemia was seen in 62.3% (38/61) of the eyes checked. Neovascularization was observed in one eye. The frequency of bilateral changes in angiography was high. Non-proliferative retinopathy was more common, especially vascular tortuosities (17/61), followed by arteriovenous anastomoses in the retinal periphery (15/61) and arterial occlusions (5/61). The mean age of retinopathy group was 14.4 years old, significantly lower than the mean age of non-retinopathy group, which was 17.4. The result was normal in16.4% (10/61) of the eyes in the fundoscopy exam, while angiography showed alterations. CONCLUSIONS: All the results pointed to the conclusion that the non-proliferative retinal vascular changes are frequent and precocious in patients with sickle cell anemia (SS genotype). Fluorescein angiography is more sensitive in the diagnosis of retinopathy when compared to indirect ophthalmoscopy.

Anemia, sickle cell; Retina; Retinal diseases; Fluorescein angiography; Ophthalmoscopy


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