{"title":"Cellular-Level Assessment of Macular Development in Patients with FEVR Using Multimodal Imaging: A Prospective Cohort Study.","authors":"Yarou Hu,Xinyu Zhao,Yaling Liu,Zhen Yu,Kaixuan Cui,Zhenquan Wu,Ziying Zhou,Ruiheng Zhang,Li Dong,Wenbin Wei,Shaochong Zhang,Wei Chi,Jie Zhang,Peiquan Zhao,Guoming Zhang","doi":"10.1016/j.ajo.2025.09.036","DOIUrl":null,"url":null,"abstract":"PURPOSE\r\nTo investigate the multimodal characteristics of macula in patients with familial exudative vitreoretinopathy (FEVR) at a cellular level.\r\n\r\nDESIGN\r\nProspective cohort study.\r\n\r\nPARTICIPANTS\r\nA total of 46 FEVR-affected and healthy eyes were enrolled from July to October 2024 and underwent multimodal examinations. Participants with conditions such as foveal hypoplasia (FH), a history of ocular surgery or trauma in both the control and FEVR groups were excluded.\r\n\r\nMETHODS AND MAIN OUTCOME MEASURES\r\nAll participants were underwent examinations of scanning laser ophthalmoscopy (SLO), ultra-wide-field swept source optical coherence tomography angiography (UWF SS-OCTA), and adaptive optics (AO)-SLO to acquire metrics of multimodal macular characteristics such as macular appearance, layered structure, vasculature and photoreceptor distribution. The baseline information such as best-corrected visual acuity (BCVA), spherical equivalent (SE), and axial length (AL) were also measured.\r\n\r\nRESULTS\r\nComparing to age (9.76 years on average), gender (52% femal), SE (-1.01D on average) and AL (23.66mm on average)-matched healthy eyes, FEVR-affected eyes without FH had increased foveal avascular zone (FAZ) perimeter in deep capillary plexus (DCP), partly thickening inner retina layers and thinning outer nuclear layer, as well as partly lower vessel density of DCP (P<0.05). In cellular-level, eyes with FEVR showed numerically lower cone density and larger cone spacing in fovea, but significantly higher cone density (P=0.03) and smaller cone spacing (P=0.02) in parafovea, as well as higher cone dispersion and lower regularity (P<0.05). Foveal and parafoveal cone density in participants with low BCVA were numerically higher.\r\n\r\nCONCLUSIONS\r\nPatients with FEVR without FH may be characterized by irregular FAZ morphology and cone distribution, exhibiting lower foveal and higher parafoveal cone densities, which was not correlated with their BCVA and may serve as clinical biomarkers for diagnostic assistance in FEVR. Participants with low BCVA but morphologically normal macular structure may harbor underlying cone abnormalities, underscoring the necessity for more thorough assessment, warranting greater caution in diagnosing amblyopia until excluding the influence of cellular-level abnormalities.","PeriodicalId":7568,"journal":{"name":"American Journal of Ophthalmology","volume":"28 1","pages":""},"PeriodicalIF":4.2000,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"American Journal of Ophthalmology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.ajo.2025.09.036","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"OPHTHALMOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
PURPOSE
To investigate the multimodal characteristics of macula in patients with familial exudative vitreoretinopathy (FEVR) at a cellular level.
DESIGN
Prospective cohort study.
PARTICIPANTS
A total of 46 FEVR-affected and healthy eyes were enrolled from July to October 2024 and underwent multimodal examinations. Participants with conditions such as foveal hypoplasia (FH), a history of ocular surgery or trauma in both the control and FEVR groups were excluded.
METHODS AND MAIN OUTCOME MEASURES
All participants were underwent examinations of scanning laser ophthalmoscopy (SLO), ultra-wide-field swept source optical coherence tomography angiography (UWF SS-OCTA), and adaptive optics (AO)-SLO to acquire metrics of multimodal macular characteristics such as macular appearance, layered structure, vasculature and photoreceptor distribution. The baseline information such as best-corrected visual acuity (BCVA), spherical equivalent (SE), and axial length (AL) were also measured.
RESULTS
Comparing to age (9.76 years on average), gender (52% femal), SE (-1.01D on average) and AL (23.66mm on average)-matched healthy eyes, FEVR-affected eyes without FH had increased foveal avascular zone (FAZ) perimeter in deep capillary plexus (DCP), partly thickening inner retina layers and thinning outer nuclear layer, as well as partly lower vessel density of DCP (P<0.05). In cellular-level, eyes with FEVR showed numerically lower cone density and larger cone spacing in fovea, but significantly higher cone density (P=0.03) and smaller cone spacing (P=0.02) in parafovea, as well as higher cone dispersion and lower regularity (P<0.05). Foveal and parafoveal cone density in participants with low BCVA were numerically higher.
CONCLUSIONS
Patients with FEVR without FH may be characterized by irregular FAZ morphology and cone distribution, exhibiting lower foveal and higher parafoveal cone densities, which was not correlated with their BCVA and may serve as clinical biomarkers for diagnostic assistance in FEVR. Participants with low BCVA but morphologically normal macular structure may harbor underlying cone abnormalities, underscoring the necessity for more thorough assessment, warranting greater caution in diagnosing amblyopia until excluding the influence of cellular-level abnormalities.
期刊介绍:
The American Journal of Ophthalmology is a peer-reviewed, scientific publication that welcomes the submission of original, previously unpublished manuscripts directed to ophthalmologists and visual science specialists describing clinical investigations, clinical observations, and clinically relevant laboratory investigations. Published monthly since 1884, the full text of the American Journal of Ophthalmology and supplementary material are also presented online at www.AJO.com and on ScienceDirect.
The American Journal of Ophthalmology publishes Full-Length Articles, Perspectives, Editorials, Correspondences, Books Reports and Announcements. Brief Reports and Case Reports are no longer published. We recommend submitting Brief Reports and Case Reports to our companion publication, the American Journal of Ophthalmology Case Reports.
Manuscripts are accepted with the understanding that they have not been and will not be published elsewhere substantially in any format, and that there are no ethical problems with the content or data collection. Authors may be requested to produce the data upon which the manuscript is based and to answer expeditiously any questions about the manuscript or its authors.