Aims:This study describes vascular abnormalities in X-linked retinoschisis(XLRS)using fundus fluorescein angiography(FFA)and ultra-widefield swept-source optical coherence tomography angiography(UWF SS-OCTA)to better ...Aims:This study describes vascular abnormalities in X-linked retinoschisis(XLRS)using fundus fluorescein angiography(FFA)and ultra-widefield swept-source optical coherence tomography angiography(UWF SS-OCTA)to better understand the disease's vascular features and impact.Methods:A retrospective cross-sectional study was conducted on 26 XLRS patients(46 eyes).A comprehensive ophthalmic examination was performed,including FFA and UWF SS-OCTA.FFA abnormalities were divided into peripheral schisis-associated and optic disc-associated types.Results:The mean age of patients was 11.3±6.5 years.Macular schisis appeared in 97.8%of eyes,peripheral schisis in 89.1%,and peripheral bullous schisis(PBS)in 67.39%.Major vascular changes identified by FFA included dendritic capillary dilation/leakage(91.3%),internal residual vessel leakage(78.3%),and capillary dropout/ischemia(71.7%).Minor changes included zonal retinal pigment epithelium(RPE)proliferation(6.5%),bridging vessels(4.4%),and capillary sheathing(4.4%).peripapillary choroidal neovascularization(PPCNV)was noted in 10.9%and situs inversus of optic disc in 13.0%of eyes.Additionally,situs in versus of optic disc and zonal RPE proliferation were novel findings.Major FFA changes correlated with broader PBS(P=0.045)(P<0.001)(P=0.003).Clock hours of PBS were significant predictors for internal residual vessel leakage(OR=0.30,P=0.03).No significant correlation was found between gene mutation type and FFA abnormalities(P=0.539).Conclusions:This study highlighted the significant prevalence(95.7%)of vascular abnormalities in XLRS and emphasized the importance of combining FFA with UWF SS-OCTA for comprehensive evaluation,enhancing the understanding of XLRS pathophysiology and aiding in targeted treatment approaches.展开更多
基金supported by the Construction Project of High-Level Hospitals in Guangdong Province(303020107,303010303058)the National Natural Science Foundation of China(82271092,82401282)+1 种基金Guangdong Basic and Applied Research Foundation(2023A1515010430)Guangzhou Municipal Science and Technology Key Project(2024A03J0171).
文摘Aims:This study describes vascular abnormalities in X-linked retinoschisis(XLRS)using fundus fluorescein angiography(FFA)and ultra-widefield swept-source optical coherence tomography angiography(UWF SS-OCTA)to better understand the disease's vascular features and impact.Methods:A retrospective cross-sectional study was conducted on 26 XLRS patients(46 eyes).A comprehensive ophthalmic examination was performed,including FFA and UWF SS-OCTA.FFA abnormalities were divided into peripheral schisis-associated and optic disc-associated types.Results:The mean age of patients was 11.3±6.5 years.Macular schisis appeared in 97.8%of eyes,peripheral schisis in 89.1%,and peripheral bullous schisis(PBS)in 67.39%.Major vascular changes identified by FFA included dendritic capillary dilation/leakage(91.3%),internal residual vessel leakage(78.3%),and capillary dropout/ischemia(71.7%).Minor changes included zonal retinal pigment epithelium(RPE)proliferation(6.5%),bridging vessels(4.4%),and capillary sheathing(4.4%).peripapillary choroidal neovascularization(PPCNV)was noted in 10.9%and situs inversus of optic disc in 13.0%of eyes.Additionally,situs in versus of optic disc and zonal RPE proliferation were novel findings.Major FFA changes correlated with broader PBS(P=0.045)(P<0.001)(P=0.003).Clock hours of PBS were significant predictors for internal residual vessel leakage(OR=0.30,P=0.03).No significant correlation was found between gene mutation type and FFA abnormalities(P=0.539).Conclusions:This study highlighted the significant prevalence(95.7%)of vascular abnormalities in XLRS and emphasized the importance of combining FFA with UWF SS-OCTA for comprehensive evaluation,enhancing the understanding of XLRS pathophysiology and aiding in targeted treatment approaches.
