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 inversusof 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 = 1.000)(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.

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 inversusof 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 = 1.000)(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.

目的：探讨应用数字化广域成像系统RetCam₃行婴幼儿口服荧光素钠眼底血管荧光造影(fluorescein fundus angiography，FFA)的护理。方法：选择2018年8月至2019年12月在广州中山大学中山眼科中心小儿眼病综合科就诊的眼底疾病婴幼儿78例，应用RetCam₃进行口服法FFA检查及护理，将护理要点进行总结。结果：所有患儿安全、顺利完成检查，检查过程中均未发生异常病情变化或与检查、药物相关的并发症。经FFA确诊家族性渗出性玻璃体视网膜病变(familial exudative vitreoretinopathy ，FEVR)26例，早产儿视网膜病变(retinopathy of prematurity ，ROP)23例，色素失禁症患者6例；玻璃体积血患者3例；视网膜母细胞瘤患者3例；牵牛花综合征患者1例；视网膜色素变性患者3例；弓蛔虫眼病患者1例；原始永存玻璃体患者2例；不明原因眼底病变患者5例，单眼视网膜皱襞患者1例，先天性小眼球患者1例，巨细胞病毒感染患者1例，先天性黄斑发育不良患者1例；Coats病患者1例。结论：应用RetCam₃行婴幼儿口服法FFA是一种安全、有效的检查方法。规范、恰当的护理配合能够保证检查准确、顺利地完成。

Objective: To share the nursing experience of RetCam₃ ultra-widefield oral fluorescein fundus angiography (FFA) in infants with fundus diseases. Methods: Seventy-eight infants with fundus diseases admitted to General Department of Pediatric Ophthalmology in Zhongshan Ophthalmic Center, Sun Yat-sen University from August 2018 to December 2019 were recruited. Oral FFA was carried out using the 130-degree lens of RetCam₃, and the key points of nursing were summarized. Results: No complications related to the examination and drugs occurred after oral FFA with an appropriate nursing manner. FFA confirmed 26 cases of familial exudative vitreoretinopathy,23 cases of retinopathy of prematurity and 6 cases of pigment incontinence. Vitreous hematoma was observed in 3 patients, retinoblastoma in 3 patients, Morning Glory syndrome in 1 patient, retinitis pigmentosa in 3 patients,Ascaris lumbricoides eye disease in 1 case, original permanent vitreous body in 2 patients, unexplained fundus lesions in 5 patients, monocular retinal fold in 1 patient, congenital micro-eyeball in 1 patient, cytomegalovirus infection in 1 patient, congenital macular dysplasia 1 patient and Coats disease in 1 patient. Conclusion: Oral FFA with RetCam₃ is an effective and safe detection method for infants. Standard and proper nursing can ensure the examination can be performed accurately and smoothly.

目的：探讨应用数字化广域成像系统RetCam3行婴幼儿口服荧光素钠眼底血管荧光造影(fluorescein fundus angiography，FFA)的护理。方法：选择2018年8月至2019年12月在广州中山大学中山眼科中心小儿眼病综合科就诊的眼底疾病婴幼儿78例，应用RetCam3进行口服法FFA检查及护理，将护理要点进行总结。结果：所有患儿安全、顺利完成检查，检查过程中均未发生异常病情变化或与检查、药物相关的并发症。经FFA确诊家族性渗出性玻璃体视网膜病变(familial exudative vitreoretinopathy ，FEVR)26例，早产儿视网膜病变(retinopathy of prematurity ，ROP)23例，色素失禁症患者6例；玻璃体积血患者3例；视网膜母细胞瘤患者3例；牵牛花综合征患者1例；视网膜色素变性患者3例；弓蛔虫眼病患者1例；原始永存玻璃体患者2例；不明原因眼底病变患者5例，单眼视网膜皱襞患者1例，先天性小眼球患者1例，巨细胞病毒感染患者1例，先天性黄斑发育不良患者1例；Coats病患者1例。结论：应用RetCam3行婴幼儿口服法FFA是一种安全、有效的检查方法。规范、恰当的护理配合能够保证检查准确、顺利地完成。

Objective: To share the nursing experience of RetCam3 ultra-widefield oral fluorescein fundus angiography (FFA) in infants with fundus diseases. Methods: Seventy-eight infants with fundus diseases admitted to General Department of Pediatric Ophthalmology in Zhongshan Ophthalmic Center, Sun Yat-sen University from August 2018 to December 2019 were recruited. Oral FFA was carried out using the 130-degree lens of RetCam3, and the key points of nursing were summarized. Results: No complications related to the examination and drugs occurred after oral FFA with an appropriate nursing manner. FFA confirmed 26 cases of familial exudative vitreoretinopathy, 23 cases of retinopathy of prematurity and 6 cases of pigment incontinence. Vitreous hematoma was observed in 3 patients, retinoblastoma in 3 patients, Morning Glory syndrome in 1 patient, retinitis pigmentosa in 3 patients, Ascaris lumbricoides eye disease in 1 case, original permanent vitreous body in 2 patients, unexplained fundus lesions in 5 patients, monocular retinal fold in 1 patient, congenital micro-eyeball in 1 patient, cytomegalovirus infection in 1 patient, congenital macular dysplasia 1 patient and Coats disease in 1 patient. Conclusion: Oral FFA with RetCam3 is an effective and safe detection method for infants. Standard and proper nursing can ensure the examination can be performed accurately and smoothly.