Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis
Purpose: To assess longitudinal changes in retinal thickness and vessel density (VD) in pediatric sickle cell disease (SCD). Design: A prospective cohort study. Participants: Children (<18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes were enrolled from a university-ba...
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Elsevier
2025-11-01
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| Series: | Ophthalmology Science |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666914525001605 |
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| author | Sally S. Ong, MD Ann Nampomba, MS Sara Rahman, BS Loka Thangamathesvaran, MD Grace Reilly, MD Jianqiao Ma, ScM Jay Vaidya, PhD Sophie Lanzkron, MD, MHS Adrienne W. Scott, MD |
| author_facet | Sally S. Ong, MD Ann Nampomba, MS Sara Rahman, BS Loka Thangamathesvaran, MD Grace Reilly, MD Jianqiao Ma, ScM Jay Vaidya, PhD Sophie Lanzkron, MD, MHS Adrienne W. Scott, MD |
| author_sort | Sally S. Ong, MD |
| collection | DOAJ |
| description | Purpose: To assess longitudinal changes in retinal thickness and vessel density (VD) in pediatric sickle cell disease (SCD). Design: A prospective cohort study. Participants: Children (<18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes were enrolled from a university-based retina subspecialty clinic from 2017 to 2019 and followed for ≥2 years. Methods: Participants received 3 × 3 and 6 × 6 mm OCT and OCT angiography scans at baseline and at each follow-up visit. Main Outcome Measures: Retinal thickness, superficial capillary plexus (SCP), and deep capillary plexus (DCP) VD were compared over time. Results: Children with HbSS (n = 14) and HbS variant (n = 14) genotypes with ≥1 follow-up were included in the study (total 56 eyes). For HbSS, rates of retinal thinning per year were significant in the inner retina in the nasal, inferior, and total parafovea (P = 0.002, 0.003, and 0.03 respectively), temporal and total perifovea (P = 0.01 and 0.02); in the middle retina in the superior perifovea (P = 0.04); and in the total retina in the superior, nasal, and total perifovea (P < 0.001, = 0.01, and 0.009). For HbS variant, the rate of retinal thinning was significant in the inner retina in the superior parafovea (P = 0.002) only. Vessel density did not change in the SCP in HbSS subjects in any of the subfields studied but increased significantly in the SCP in HbS variant subjects in the nasal and inferior parafovea (P = 0.02 and 0.045) and superior and nasal perifovea (P = 0.03 and 0.004). Vessel density in the DCP increased in the HbSS group in all the subfields studied (P < 0.05) and in the HbS variant group in the temporal parafovea (P = 0.02). Conclusions: Progressive retinal thinning, predominantly in the inner retinal layers, was particularly notable in children with HbSS disease. This was observed in conjunction with the lack of an increase in the SCP VD in the HbSS group when compared with the HbS variant group. These findings suggest that microstructural and microvasculature abnormalities in the macula start in childhood in SCD, especially for those with HbSS disease. Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article. |
| format | Article |
| id | doaj-art-782f6ac442f645039fccfd3cbb0b473d |
| institution | DOAJ |
| issn | 2666-9145 |
| language | English |
| publishDate | 2025-11-01 |
| publisher | Elsevier |
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| series | Ophthalmology Science |
| spelling | doaj-art-782f6ac442f645039fccfd3cbb0b473d2025-08-20T02:48:39ZengElsevierOphthalmology Science2666-91452025-11-015610086210.1016/j.xops.2025.100862Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal AnalysisSally S. Ong, MD0Ann Nampomba, MS1Sara Rahman, BS2Loka Thangamathesvaran, MD3Grace Reilly, MD4Jianqiao Ma, ScM5Jay Vaidya, PhD6Sophie Lanzkron, MD, MHS7Adrienne W. Scott, MD8Department of Ophthalmology, Wake Forest School of Medicine, Winston-Salem, North Carolina; Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MarylandRetina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MarylandRetina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MarylandRetina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MarylandDepartment of Ophthalmology, University of North Carolina-Chapel Hill, Chapel Hill, North CarolinaBiostatistics, Epidemiology and Data Management (BEAD) Core, Johns Hopkins University School of Medicine, Baltimore, MarylandBiostatistics, Epidemiology and Data Management (BEAD) Core, Johns Hopkins University School of Medicine, Baltimore, MarylandDivision of Hematology, Thomas Jefferson University, Philadelphia, PennsylvaniaRetina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland; Correspondence: Adrienne W. Scott, MD, Retina Division, Wilmer Eye Institute, 600 N Wolfe St, Maumenee 719, Baltimore, MD 21287.Purpose: To assess longitudinal changes in retinal thickness and vessel density (VD) in pediatric sickle cell disease (SCD). Design: A prospective cohort study. Participants: Children (<18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes were enrolled from a university-based retina subspecialty clinic from 2017 to 2019 and followed for ≥2 years. Methods: Participants received 3 × 3 and 6 × 6 mm OCT and OCT angiography scans at baseline and at each follow-up visit. Main Outcome Measures: Retinal thickness, superficial capillary plexus (SCP), and deep capillary plexus (DCP) VD were compared over time. Results: Children with HbSS (n = 14) and HbS variant (n = 14) genotypes with ≥1 follow-up were included in the study (total 56 eyes). For HbSS, rates of retinal thinning per year were significant in the inner retina in the nasal, inferior, and total parafovea (P = 0.002, 0.003, and 0.03 respectively), temporal and total perifovea (P = 0.01 and 0.02); in the middle retina in the superior perifovea (P = 0.04); and in the total retina in the superior, nasal, and total perifovea (P < 0.001, = 0.01, and 0.009). For HbS variant, the rate of retinal thinning was significant in the inner retina in the superior parafovea (P = 0.002) only. Vessel density did not change in the SCP in HbSS subjects in any of the subfields studied but increased significantly in the SCP in HbS variant subjects in the nasal and inferior parafovea (P = 0.02 and 0.045) and superior and nasal perifovea (P = 0.03 and 0.004). Vessel density in the DCP increased in the HbSS group in all the subfields studied (P < 0.05) and in the HbS variant group in the temporal parafovea (P = 0.02). Conclusions: Progressive retinal thinning, predominantly in the inner retinal layers, was particularly notable in children with HbSS disease. This was observed in conjunction with the lack of an increase in the SCP VD in the HbSS group when compared with the HbS variant group. These findings suggest that microstructural and microvasculature abnormalities in the macula start in childhood in SCD, especially for those with HbSS disease. Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.http://www.sciencedirect.com/science/article/pii/S2666914525001605sickle cell retinopathychildrenOCTAretinal thinningvessel density |
| spellingShingle | Sally S. Ong, MD Ann Nampomba, MS Sara Rahman, BS Loka Thangamathesvaran, MD Grace Reilly, MD Jianqiao Ma, ScM Jay Vaidya, PhD Sophie Lanzkron, MD, MHS Adrienne W. Scott, MD Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis Ophthalmology Science sickle cell retinopathy children OCTA retinal thinning vessel density |
| title | Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis |
| title_full | Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis |
| title_fullStr | Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis |
| title_full_unstemmed | Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis |
| title_short | Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis |
| title_sort | macular thinning and microvasculature abnormalities in children with sickle cell disease a longitudinal analysis |
| topic | sickle cell retinopathy children OCTA retinal thinning vessel density |
| url | http://www.sciencedirect.com/science/article/pii/S2666914525001605 |
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