First Author: M.Mota PORTUGAL
Co Author(s): D. Silva M. Ramalho F. Silva S. Teixeira G. Pires I. Prieto
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To evaluate the macular alterations observed by optical coherence tomography (OCT), in children with sickle cell disease (SCD).
Ophthalmology Department, Hospital Prof. Dr. Fernando Fonseca E.P.E., Amadora, Portugal.
Cross-sectional study that included two groups, one with 28 eyes of 14 patients (mean age: 14,36 years 4,466) with SCD and a control group, with the same number of children (mean age: 12,45 years 5,648). Patients with SCD were divided into two subgroups, one with and one without sickle cell retinopathy. All patients had the SS genotype. Both groups underwent OCT for evaluating macular thickness, we also perform OCT using the enhanced depth imaging (EDI) program, for choroidal thickness evaluation. All participants in this study had better corrected visual acuity of 10/10, and had no other known diseases, neither major refractive defects. Tests were considered significant for a significance level of α=0.05.
In children with SCD, 10 eyes with retinopathy were observed, being proliferative in 6 eyes (maximum stage: II). We did not observe significant differences in macular thickness and choroidal thickness between two groups studied. However, in two eyes, of different children with SCD, we observe marked thinning of the retina in the temporal macula, change not found in the control group. One of these patients had proliferative retinopathy but, the other patient, did not present changes compatible with retinopathy in the ocular fundus exam.
Although children with SCD did not present a macular thickness decrease significantly different from the control group, some of them present alterations only described in adults, to date. This alteration (marked thinning of the retina in the temporal macula) presumably reflect the chronic ischaemia of the inner layers of the retina and an asymptomatic microvascular disease. The fact that we found this change in one child without identifiable retinopathy in the ocular fund, indicates to us that this isolated examination may not be enough to define the existence of retinopathy. We conclude that the OCT should be mandatory for retinopathy screening in these patients, even in paediatric age. Further studies are necessary to detect a possible association between the temporal macular thinning and a more severe illness in adult life.