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Abstract：
OBJECTIVE: To review the current sickle cell disease (SCD) literature to assess how \"retinopathy\" has been defined and to identify ocular outcomes that have been measured and described.
METHODS: A systematic scoping review of SCD literature was completed regarding ocular manifestations of SCD and vision outcomes across all medical specialties.
METHODS: Participants with SCD and control patients were included in our data extraction.
METHODS: We reviewed English-language literature from 2000 to 2021 for eligible studies by searching PubMed, Google Scholar, Embase, and the Cochrane library using terms to encompass SCD and ocular findings.
METHODS: Data collection included study information, patient characteristics, vision-related findings (inclusion criteria and/or study outcomes), and retinopathy characteristics (definition, when, how and by whom diagnosed).
RESULTS: We identified 4006 unique citations and 111 were included in the analysis. Ophthalmologists were senior authors of about half (59/111; 53.2%) of the articles; most articles were published between 2016 and 2021 (71/111; 70.0%). The studies had been conducted primarily in North America (54/111; 48.6%) or Europe (23/111; 20.7%); designs were cross-sectional (51/111; 45.9%), prospective cohort (28/111; 25.2%), retrospective cohort (27/111; 24.3%), and case-control (4/111; 3.6%). Among studies reporting any retinopathy, it was commonly defined as a combination of nonproliferative sickle cell retinopathy and proliferative sickle cell retinopathy (PSR; 52/87; 59.8%), infrequently as PSR only (6/87; 6.9%), or not defined at all (23/87; 26.4%). The Goldberg classification was used to grade retinopathy in almost half of the studies (41/87; 47.1%). Investigators reporting diagnostic methods used clinical fundus examination (56/111; 50.4%), OCT (24/111; 21.6%), fluorescein angiography (20/111; 18.0%), ultrawidefield fundus photographs (15/111; 13.5%), and OCT angiography (10/111; 9.0%), or did not report methods (28/111; 25.2%).
CONCLUSIONS: There are inconsistencies in documentation of methods and outcomes in studies of SCD ophthalmic findings. Particularly concerning is the lack of documentation of ophthalmic examination methods, qualifications of examiners, and clarity and specificity of sickle cell retinopathy definitions. With the increase in SCD treatment research and novel systemic therapies available, it is important to adopt clear and consistent descriptions and rigorous data collection and reporting of ophthalmic outcomes in SCD studies.
BACKGROUND: The authors have no proprietary or commercial interest in any materials discussed in this article.
METHODS: A systematic scoping review of SCD literature was completed regarding ocular manifestations of SCD and vision outcomes across all medical specialties.
METHODS: Participants with SCD and control patients were included in our data extraction.
METHODS: We reviewed English-language literature from 2000 to 2021 for eligible studies by searching PubMed, Google Scholar, Embase, and the Cochrane library using terms to encompass SCD and ocular findings.
METHODS: Data collection included study information, patient characteristics, vision-related findings (inclusion criteria and/or study outcomes), and retinopathy characteristics (definition, when, how and by whom diagnosed).
RESULTS: We identified 4006 unique citations and 111 were included in the analysis. Ophthalmologists were senior authors of about half (59/111; 53.2%) of the articles; most articles were published between 2016 and 2021 (71/111; 70.0%). The studies had been conducted primarily in North America (54/111; 48.6%) or Europe (23/111; 20.7%); designs were cross-sectional (51/111; 45.9%), prospective cohort (28/111; 25.2%), retrospective cohort (27/111; 24.3%), and case-control (4/111; 3.6%). Among studies reporting any retinopathy, it was commonly defined as a combination of nonproliferative sickle cell retinopathy and proliferative sickle cell retinopathy (PSR; 52/87; 59.8%), infrequently as PSR only (6/87; 6.9%), or not defined at all (23/87; 26.4%). The Goldberg classification was used to grade retinopathy in almost half of the studies (41/87; 47.1%). Investigators reporting diagnostic methods used clinical fundus examination (56/111; 50.4%), OCT (24/111; 21.6%), fluorescein angiography (20/111; 18.0%), ultrawidefield fundus photographs (15/111; 13.5%), and OCT angiography (10/111; 9.0%), or did not report methods (28/111; 25.2%).
CONCLUSIONS: There are inconsistencies in documentation of methods and outcomes in studies of SCD ophthalmic findings. Particularly concerning is the lack of documentation of ophthalmic examination methods, qualifications of examiners, and clarity and specificity of sickle cell retinopathy definitions. With the increase in SCD treatment research and novel systemic therapies available, it is important to adopt clear and consistent descriptions and rigorous data collection and reporting of ophthalmic outcomes in SCD studies.
BACKGROUND: The authors have no proprietary or commercial interest in any materials discussed in this article.
摘要：
目的：回顾当前的镰状细胞病（SCD）文献，以评估“视网膜病变”的定义，并确定已测量和描述的眼部结局。
方法：完成了关于SCD眼部表现和所有医学专业视力结果的SCD文献的系统范围综述。
方法：SCD患者和对照患者纳入我们的数据提取。
方法：我们通过搜索PubMed，回顾了2000-2021年的英语文献中符合条件的研究，谷歌学者，Embase,和Cochrane库使用术语涵盖SCD和眼部发现。
方法：数据收集包括研究信息，患者特征,与视力相关的发现（纳入标准和/或研究结果），和视网膜病变特征(定义，when,如何以及由谁诊断)。
结果：我们确定了4,006个独特的引文和111个被纳入分析。眼科医生是大约一半(59/111;53.2%)文章的高级作者;大多数文章发表于2016年至2021年(71/111;70.0%)。这些研究主要在北美（54/111;48.6％）或欧洲（23/111;20.7％）进行；设计是横截面的（51/111;45.9％），前瞻性队列（28/111；25.2％），回顾性队列(27/111;24.3%)和病例对照(4/111;3.6%).在报告任何视网膜病变的研究中，它通常被定义为非增生性镰状细胞视网膜病变（NPSR）和增生性镰状细胞视网膜病变（PSR）的组合（52/87;59.8％），很少作为PSR（6/87；6.9%），或根本没有定义（23/87；26.4%）。在几乎一半的研究中（41/87；47.1％）使用Goldberg分类法对视网膜病变进行分级。报告诊断方法的研究者使用临床眼底检查（56/111;50.4%），光学相干断层扫描（OCT）（24/111；21.6%），荧光素血管造影（20/111;18.0%），超宽视野眼底照片（15/111；13.5％），和OCT血管造影（OCTA）（10/111;9.0％），或未报告方法（28/111；25.2%）。
结论：SCD眼科发现的方法和结果文献记录存在不一致之处。特别令人担忧的是缺乏眼科检查方法的文件，考官的资格，以及镰状细胞视网膜病变定义的清晰度和特异性。随着SCD治疗研究和新的全身疗法的增加，在SCD研究中,对眼科结局采用清晰一致的描述,严格的数据收集和报告非常重要.
方法：完成了关于SCD眼部表现和所有医学专业视力结果的SCD文献的系统范围综述。
方法：SCD患者和对照患者纳入我们的数据提取。
方法：我们通过搜索PubMed，回顾了2000-2021年的英语文献中符合条件的研究，谷歌学者，Embase,和Cochrane库使用术语涵盖SCD和眼部发现。
方法：数据收集包括研究信息，患者特征,与视力相关的发现（纳入标准和/或研究结果），和视网膜病变特征(定义，when,如何以及由谁诊断)。
结果：我们确定了4,006个独特的引文和111个被纳入分析。眼科医生是大约一半(59/111;53.2%)文章的高级作者;大多数文章发表于2016年至2021年(71/111;70.0%)。这些研究主要在北美（54/111;48.6％）或欧洲（23/111;20.7％）进行；设计是横截面的（51/111;45.9％），前瞻性队列（28/111；25.2％），回顾性队列(27/111;24.3%)和病例对照(4/111;3.6%).在报告任何视网膜病变的研究中，它通常被定义为非增生性镰状细胞视网膜病变（NPSR）和增生性镰状细胞视网膜病变（PSR）的组合（52/87;59.8％），很少作为PSR（6/87；6.9%），或根本没有定义（23/87；26.4%）。在几乎一半的研究中（41/87；47.1％）使用Goldberg分类法对视网膜病变进行分级。报告诊断方法的研究者使用临床眼底检查（56/111;50.4%），光学相干断层扫描（OCT）（24/111；21.6%），荧光素血管造影（20/111;18.0%），超宽视野眼底照片（15/111；13.5％），和OCT血管造影（OCTA）（10/111;9.0％），或未报告方法（28/111；25.2%）。
结论：SCD眼科发现的方法和结果文献记录存在不一致之处。特别令人担忧的是缺乏眼科检查方法的文件，考官的资格，以及镰状细胞视网膜病变定义的清晰度和特异性。随着SCD治疗研究和新的全身疗法的增加，在SCD研究中,对眼科结局采用清晰一致的描述,严格的数据收集和报告非常重要.
