Brandsen Rajani P, Biemond Bart J, Nur Erfan, Chang-Wolf Jennifer M, Schlingemann Reinier O, Diederen Roselie M H
Departments of Ophthalmology, and.
Hematology, Amsterdam UMC, Amsterdam, the Netherlands.
Retina. 2025 May 1;45(5):928-938. doi: 10.1097/IAE.0000000000004393. Epub 2025 Jan 2.
To evaluate the presence and progression of maculopathy in patients with sickle cell disease using optical coherence tomography and optical coherence tomography-angiography and to identify clinical/laboratory risk factors for progression during follow-up.
Complete ophthalmic examination, including fundoscopy and macular spectral-domain-optical coherence tomography/optical coherence tomography angiography scans, was performed in consecutive patients with sickle cell disease (HbSS/HbSβ 0 /HbSβ + /HbSC genotype) during baseline and follow-up visits. Sickle cell retinopathy stage was based on fundoscopy instead of the Goldberg classification, as fluorescein angiography was not routinely used. Medical/ophthalmological history and hematologic characteristics were retrieved from medical records.
One hundred and six eyes of 60 patients were analyzed. The median follow-up period was 34.5 months (range 8-70, interquartile range 25-55). Macular thinning was present in 41 eyes (38.7%) at baseline and in 52 eyes (49.1%) at follow-up. Progression of macular thinning was observed in 25.5% (27/106) of the eyes and sickle cell retinopathy progression in 15.1% (16/106) of the eyes. Predictors for the progression of macular thinning were proliferative retinopathy (adjusted odds ratio 3.40, P = 0.024), lower vessel density in the superior capillary plexus of the inferior parafoveal subfield (adjusted odds ratio 0.88, P = 0.003), and higher vessel density in the deep capillary plexus of the inferior parafoveal subfield (adjusted odds ratio 1.17, P = 0.001). No association was found between the progression of macular thinning and the worsening of other organ damage, sickle cell retinopathy progression, ocular complications, or laser treatment.
Sickle cell disease-related maculopathy progresses in many patients without impairing visual acuity during short-term follow-up. Progression of maculopathy is correlated with proliferative retinopathy and vessel densities in inferior parafoveal subfields. Further research is needed to elucidate functional consequences of macular changes.
使用光学相干断层扫描（OCT）和光学相干断层扫描血管造影（OCTA）评估镰状细胞病患者黄斑病变的存在和进展情况，并确定随访期间进展的临床/实验室危险因素。
对连续的镰状细胞病（HbSS/HbSβ0/HbSβ+/HbSC基因型）患者在基线和随访期间进行全面眼科检查，包括眼底镜检查和黄斑区光谱域光学相干断层扫描/光学相干断层扫描血管造影扫描。镰状细胞视网膜病变分期基于眼底镜检查而非戈德堡分类，因为荧光素血管造影未常规使用。从病历中获取患者的医学/眼科病史和血液学特征。
分析了60例患者的106只眼。中位随访期为34.5个月（范围8 - 70个月，四分位间距25 - 55个月）。基线时41只眼（38.7%）存在黄斑变薄，随访时52只眼（49.1%）存在黄斑变薄。25.5%（27/106）的眼观察到黄斑变薄进展，15.1%（16/106）的眼观察到镰状细胞视网膜病变进展。黄斑变薄进展的预测因素为增殖性视网膜病变（调整优势比3.40，P = 0.024）、下方黄斑旁子区域上方毛细血管丛血管密度降低（调整优势比0.88，P = 0.003）以及下方黄斑旁子区域深层毛细血管丛血管密度升高（调整优势比1.17，P = 0.001）。未发现黄斑变薄进展与其他器官损害加重、镰状细胞视网膜病变进展、眼部并发症或激光治疗之间存在关联。
在短期随访中，许多镰状细胞病相关黄斑病变患者病情进展但未损害视力。黄斑病变进展与增殖性视网膜病变及下方黄斑旁子区域的血管密度相关。需要进一步研究以阐明黄斑变化的功能后果。
