Sickle Cell Retinopathy: Characterization of a Pediatric Population by Multimodal Imaging
DOI:
https://doi.org/10.48560/rspo.25974Keywords:
Anemia, Sickle Cell/complications, Eye Manifestations, Multimodal Imaging, Retinal Diseases/diagnosisAbstract
INTRODUCTION: Retinopathy is the most frequent ophthalmological manifestation of sickle cell disease (SCD). Advances in retinal multimodal imaging have revealed the presence of retinopathy in asymptomatic patients. The present study aims to characterize the typical retinal changes by multimodal imaging in a pediatric population with SCD, allowing the non-invasive observation of the vascular effects of the disease.
MATERIAL AND METHODS: Observational study that included children diagnosed with SCD referred from the Pediatric Hematology consultation at Hospital Garcia de Orta. The evaluation consisted of a complete ophthalmologic evaluation and multimodal imaging: wide-field fundus photography (Zeiss CLARUS 700), optical coherence tomography (OCT) and OCT- angiography (OCT-A) (Zeiss Cirrus HD-OCT 5000), with particular interest in the assessment of the vascular zone area and superficial macular vascular flow and density.
RESULTS: Our study included 28 children with SCD, mean age 12.33 ± 3.19 years, 16 (57%) female and 12 (43%) male. The most frequent genotype was SS (26 patients). Of the 56 eyes evalu- ated, 20 (36%) had non-proliferative retinopathy and 1 (1.8%) had proliferative retinopathy. The most frequently detected retinal change was the sunburst lesion (9 eyes, 16%). Also noteworthy is the presence of dark without pressure lesions in 4 eyes (2%). There was a statistically significant difference (p<0.05) between external macular superficial vascular density and the presence of retinopathy.
CONCLUSION: The present study revealed that the external macular superficial vascular density was lower in patients with retinopathy. On the other hand, the most frequent retinal finding was the sunburst lesion, in line with what was reported in previous studies.
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