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Funduscopic alterations in SS and SC hemoglobinopathies. Study of a Brazilian population
M T Bonanomi1, S L Cunha, J T de Araújo
1Department of Ophthalmology, São Paulo University Medical School, Brazil.
Insights
Sickle cell retinopathy presents differently between SS and SC hemoglobinopathy types. SC patients show more frequent proliferative retinopathy and vision loss compared to SS patients, who had more atrophic retinal tears.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is a group of inherited red blood cell disorders.
- Retinopathy is a common complication of SCD, affecting vision.
- Different SCD genotypes may influence the type and severity of retinopathy.
Purpose of the Study:
- To compare fundus changes in Brazilian patients with sickle cell retinopathy.
- To investigate differences between SS and SC hemoglobinopathy types regarding ophthalmoscopic findings.
Main Methods:
- Retrospective study of 63 Brazilian patients with sickle cell retinopathy.
- Analysis of fundus changes in patients with SS hemoglobinopathy (41) and SC hemoglobinopathy (22).
- Comparison of ophthalmoscopic and angiographic findings between the two groups.
Main Results:
- Ophthalmoscopic changes were more frequent in the SC hemoglobinopathy group than in the SS group.
- Atrophic retinal tears were exclusively observed in the SS group.
- Proliferative retinopathy and its complications occurred in 54.54% of SC eyes versus 14.64% of SS eyes.
- Greater vision decrease was noted in the SC group.
Conclusions:
- Sickle cell retinopathy manifestations differ significantly between SS and SC hemoglobinopathy.
- SC hemoglobinopathy is associated with a higher prevalence of proliferative retinopathy and vision impairment.
- While funduscopic changes are common, severe irreversible vision loss is rare, as exemplified by one case of unsuccessful retinal detachment surgery.
Abstract:
Fundus changes in sickle cell disease are well studied and documented according to their clinical and angiographic aspects. Sickle cell retinopathy was studied in a Brazilian population of 63 patients (41 with SS hemoglobinopathy, and 22 with SC hemoglobinopathy). All ophthalmoscopic changes observed in our patients were more frequent in the SC group with the exception of atrophic retinal tears, found only in the SS group. Proliferative retinopathy including its complications was seen in 54.54% of the eyes of the SC group, and in only 14.64% of the eyes of the SS group. Decreased vision is consequently greater in the SC group. Despite the great frequency of funduscopic changes in both groups, only 1 patient suffered irreversible visual loss due to retinal detachment operated on without success.
