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Multimodal Volumetric Retinal Imaging by Oblique Scanning Laser Ophthalmoscopy oSLO and Optical Coherence Tomography OCT
Published on: August 4, 2018
Retinal Thickness and Microvascular Changes in Children With Sickle Cell Disease Evaluated by Optical Coherence
Sally S Ong1, Marguerite O Linz1, Ximin Li2
1Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Insights
Children with sickle cell disease (SCD) exhibit reduced retinal vasculature density compared to controls. Microvascular changes in SCD may occur before observable retinal thinning.
Area of Science:
- Ophthalmology
- Hematology
- Medical Imaging
Background:
- Sickle cell disease (SCD) is a genetic blood disorder that can affect various organs, including the eyes.
- Macular vascular changes are a known complication of SCD, but their progression in pediatric patients requires further elucidation.
Purpose of the Study:
- To compare the severity of macular vascular changes in children with sickle cell disease (SCD) against age- and race-matched controls.
- To assess differences in retinal thickness, vessel density (VD), and foveal avascular zone (FAZ) size between SCD genotypes and controls.
Main Methods:
- A cross-sectional study involving children (<18 years) with HbSS and HbS variant genotypes and matched controls.
- Optical coherence tomography angiography (OCTA) scans were performed to measure retinal thickness, VD in the superficial (SCP) and deep (DCP) capillary plexuses, and FAZ size.
Main Results:
- Total VD was significantly lower in HbS variant eyes (SCP and DCP) and in HbSS eyes (DCP) compared to controls.
- A higher proportion of HbSS eyes showed pathologic retinal thinning with associated SCP and DCP flow loss compared to HbS variant eyes.
- Retinal thickness and FAZ size did not significantly differ between SCD groups and controls.
Conclusions:
- Children with SCD demonstrate reduced retinal vasculature density on OCTA compared to controls, despite similar retinal thickness.
- These findings suggest that microvascular insult in SCD may precede structural retinal changes.
- OCTA is a valuable tool for detecting early vascular alterations in pediatric SCD patients.
Purpose:
To compare the severity of macular vascular changes in children with sickle cell disease (SCD) vs age- and race-matched controls.
Design:
Cross-sectional study.
Methods:
Children (<18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes, and their age- and race-matched controls, were recruited between January 2017 and December 2018. All subjects underwent optical coherence tomography angiography (OCTA) scans centered on the fovea and temporal macula. Retinal thickness, superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) size were measured and compared between HbSS and HbS variant vs controls.
Results:
Thirty-four HbSS, 34 HbS variant (Goldberg staging 0-3 for SCD eyes), and 24 control eyes (total 48 children, aged 5-17 years) were included. Total VD (3-mm ETDRS circle) was lower in HbS variant eyes than in controls for both the SCP (42.9% vs 47.7%, P = .02) and DCP (47.4% vs 52.6%, P = .01). In HbSS eyes, VD was lower in the DCP (47.7%, P = .008) but not in the SCP (45.5%, P = .5), compared to controls. A higher proportion of HbSS (n = 18, 55%) than HbS variant eyes (n = 9, 26%) had pathologic areas of retinal thinning associated with SCP and DCP flow loss (P = .03). However, retinal thickness measurements and FAZ size did not differ between either HbSS or HbS variant group vs controls.
Conclusions:
Children with SCD have similar retinal thickness but less dense vasculature on OCTA compared to age and race-matched controls, suggesting that microvascular insult may precede structural thinning.
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