Drexel University College of Medicine, Philadelphia, Pennsylvania; Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Vanderbilt University School of Medicine, Nashville, Tennessee.
Ophthalmol Retina. 2024 Jan;8(1):81-87. doi: 10.1016/j.oret.2023.08.011. Epub 2023 Aug 25.
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.
A systematic scoping review of SCD literature was completed regarding ocular manifestations of SCD and vision outcomes across all medical specialties.
Participants with SCD and control patients were included in our data extraction.
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.
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).
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%).
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.
FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.
回顾当前镰状细胞病（SCD）文献，评估“视网膜病变”的定义，并确定已测量和描述的眼部结局。
对 SCD 文献进行了系统的范围审查，涉及 SCD 的眼部表现和所有医学专业的视力结果。
纳入了 SCD 和对照患者的参与者。
我们通过在 PubMed、Google Scholar、Embase 和 Cochrane 图书馆中搜索包含 SCD 和眼部发现的术语，对 2000 年至 2021 年的英文文献进行了合格研究的综述。
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）以及视网膜病变特征（定义、何时、如何以及由谁诊断）。
我们确定了 4006 条独特的引文，其中 111 条被纳入分析。眼科医生是大约一半（59/111；53.2%）文章的资深作者；大多数文章发表于 2016 年至 2021 年之间（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；设计为横断面（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，通常将其定义为非增生性镰状细胞性视网膜病变和增生性镰状细胞性视网膜病变的组合（52/87；59.8%），很少将其定义为仅增生性镰状细胞性视网膜病变（6/87；6.9%），或根本未定义（23/87；26.4%）。将近一半的研究（41/87；47.1%）使用 Goldberg 分类来分级视网膜病变。报告诊断方法的研究人员使用了临床眼底检查（56/111；50.4%）、OCT（24/111；21.6%）、荧光素血管造影（20/111；18.0%）、超广角眼底照片（15/111；13.5%）和 OCT 血管造影术（10/111；9.0%），或者未报告方法（28/111；25.2%）。
在 SCD 眼部发现的研究中，在方法和结果的记录方面存在不一致之处。特别令人关注的是缺乏眼科检查方法、检查人员资质以及镰状细胞性视网膜病变定义的清晰度和特异性的记录。随着 SCD 治疗研究的增加和新型全身治疗方法的出现，在 SCD 研究中采用清晰一致的描述以及严格的数据收集和报告眼部结局非常重要。
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