Department of Ophthalmology, University of Bonn, Bonn, Germany.
Nuffield Department of Clinical Neurosciences, University of Oxford, The Nuffield Laboratory of Ophthalmology, Sleep and Circadian Neuroscience Institute, Oxford, UK.
Clin Exp Ophthalmol. 2019 Mar;47(2):240-249. doi: 10.1111/ceo.13385. Epub 2018 Nov 14.
The diagnostic accuracy of different retinal imaging modalities to detect active choroidal neovascularization (CNV) in pseudoxanthoma elasticum (PXE) is essential to enable a correct diagnosis but is currently poorly understood.
Optical coherence tomography (OCT), fluorescein angiography (FA) and OCT angiography (OCT-A) are employed in daily practice, but a systematic comparison of these imaging techniques is lacking.
Retrospective, observational study.
Twenty patients (31 eyes) with PXE.
OCT, FA and OCT-A imaging was performed in each eye and graded separately by independent readers.
Diagnostic accuracy, sensitivity and specificity to detect CNV-activity of each modality and longitudinal change of CNV size measured by OCT-A.
OCT showed the highest diagnostic accuracy (kappa = 0.57) in comparison to OCT-A or FA (kappa = 0.39 and 0.37, respectively). OCT-A, OCT and FA showed a diagnostic sensitivity of 0.9, 0.85 and 0.6, and a diagnostic specificity of 0.45, 0.72 and 0.82, respectively. Evaluation of longitudinal OCT recordings (24 eyes) resulted in optimal sensitivity and specificity (kappa = 1.0). Although median CNV size assessed using OCT-A remained stable on longitudinal measures of seven eyes, two eyes showed a distinct increase over time despite anti-vascular endothelial growth factor treatment.
The systematic use of OCT, FA and OCT-A imaging can facilitate the diagnostic accuracy for detection and follow-up of CNV activity in PXE. While structural OCT is of high value, especially when longitudinal follow-up images are available, FA and OCT-A data might contribute to diagnostic accuracy in more complex cases.
不同的视网膜成像方式在诊断弹性假黄瘤（PXE）患者的活动性脉络膜新生血管（CNV）中的准确性对于正确诊断至关重要，但目前对此了解甚少。
光学相干断层扫描（OCT）、荧光素血管造影（FA）和 OCT 血管造影（OCT-A）在日常实践中被应用，但这些成像技术的系统比较尚缺乏。
回顾性、观察性研究。
20 名（31 只眼）PXE 患者。
对每只眼进行 OCT、FA 和 OCT-A 成像，并由独立的读者分别进行分级。
每种方法检测 CNV 活性的诊断准确性、敏感性和特异性，以及 OCT-A 测量的 CNV 大小的纵向变化。
与 OCT-A 或 FA 相比，OCT 显示出最高的诊断准确性（kappa=0.57）（kappa 分别为 0.39 和 0.37）。OCT-A、OCT 和 FA 的诊断敏感性分别为 0.9、0.85 和 0.6，诊断特异性分别为 0.45、0.72 和 0.82。对 24 只眼的纵向 OCT 记录进行评估，结果显示最佳的敏感性和特异性（kappa=1.0）。尽管通过纵向测量七只眼的 OCT 评估，CNV 大小的中位数保持稳定，但在接受抗血管内皮生长因子治疗的两只眼中，CNV 大小随时间明显增加。
系统使用 OCT、FA 和 OCT-A 成像可以提高 PXE 患者 CNV 活性检测和随访的诊断准确性。虽然结构 OCT 具有很高的价值，尤其是在有纵向随访图像的情况下，但 FA 和 OCT-A 数据可能有助于在更复杂的病例中提高诊断准确性。
