TY - JOUR
T1 - Retinal Thickness and Microvascular Changes in Children With Sickle Cell Disease Evaluated by Optical Coherence Tomography (OCT) and OCT Angiography
AU - Ong, Sally
AU - Linz, Marguerite O.
AU - Li, Ximin
AU - Liu, T. Y.Alvin
AU - Han, Ian
AU - Scott, Adrienne W.
N1 - Funding Information:
Funding/Support: This publication was supported through unrestricted contributions to the Johns Hopkins University Retina Division research fund, and in part by private philanthropy from Gail C. and Howard Woolley. The sponsors or funding organizations had no role in the design or conduct of this research. Financial Disclosures: None of the authors has any financial/conflicting interests to disclose. All authors attest that they meet the current ICMJE criteria for authorship. Acknowledgments: The authors gratefully thank Edward Kuwera, MD, Megan E. Collins, MD, Laura diMeglio, OD, and Jack Prince, OD from the Wilmer pediatric ophthalmology service for allowing us to recruit their unaffected patients as controls for this study.
Publisher Copyright:
© 2019 Elsevier Inc.
PY - 2020/1
Y1 - 2020/1
N2 - 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.
AB - 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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U2 - 10.1016/j.ajo.2019.08.019
DO - 10.1016/j.ajo.2019.08.019
M3 - Article
C2 - 31473216
AN - SCOPUS:85074425261
SN - 0002-9394
VL - 209
SP - 88
EP - 98
JO - American journal of ophthalmology
JF - American journal of ophthalmology
ER -