High-Resolution In Vivo Imaging in Achromatopsia
High-Resolution In Vivo Imaging in Achromatopsia
复制标题
DOI：
10.1016/j.ophtha.2010.08.053
复制
发表时间：
2011-05-01
期刊：
影响因子：
13.7
通讯作者：
Gottlob, Irene
中科院分区：
文献类型：
--
作者：
Thomas, Mervyn G.;Kumar, Anil;Gottlob, Irene
目的：应用超高分辨率(UHR)光谱域光学相干断层扫描(OCT)研究色盲患者的视网膜改变，以了解人类色视与其动物模型的对应关系。设计：对比病例系列。参与者和对照：使用超高分辨率OCT(哥白尼；OPTOPOL Technology S.A.，Zawiercie，波兰Zawiercie；3微米轴向分辨率)获得13例(26只眼)色视患者和20例(40只眼)对照组的视网膜断层图像。方法：采用三维扫描程序(743x75；AxB Scan)对中心凹中心7x7 mm的视网膜区域进行采样，获得中心凹断层图像。输出中心凹的单个B超，并使用ImageJ(Wayne Rasband，国家健康研究所)分析反射曲线和形态异常。主要观察指标：描述OCT的大体形态变化。具体地说，注意到内段和外段(IS/OS)连接和锥形外段尖端(COST)中断。结果：13例患者中有7例出现黄斑中心凹深度、外核层厚度和视网膜厚度，并与年龄呈正相关(P=0.001)。高分辨带面积不对称，鼻区明显大于颞区(P=0.002)。在所有患者中，在中心凹或黄斑旁区域或两者都有IS/OS连接中断。13名患者中有5名患者的成本反射率也出现了中断。与对照组相比，色素组的中层厚度明显变薄(P=1.1×10~(-6))，且呈年龄依赖性(P=0.0002)。13例患者中有9例出现中心凹发育不良。色素组黄斑中心凹深度(P=7.7x10(-6))和反应时(Rt)(Px1.46x10(-9))也明显低于对照组。IS/OS连接和COST反射率的破坏以及HRZ和ONL变薄的存在是视锥感光细胞退化的迹象。后两种是年龄相关的，这表明色盲是一种进行性疾病。此外，还描述了黄斑中心凹发育不良；这是与视锥感光细胞退化有关的胎儿发育缺陷。财务披露(S)：作者(S)对本文讨论的任何材料没有专利或商业利益。眼科2011；美国眼科学会118：882-887(C)2011。
Purpose: To characterize the retinal changes in patients with achromatopsia using an ultrahigh-resolution (UHR) spectral-domain optical coherence tomography (OCT) to examine how human achromatopsia corresponds to its animal model.Design: Comparative case series.Participants and Controls: Ultrahigh-resolution OCT (Copernicus; OPTOPOL Technology S.A., Zawiercie, Poland; 3-mu m axial resolution) was used to obtain scans from 13 patients (26 eyes) with achromatopsia and from 20 controls (40 eyes).Methods: A 3-dimensional scan program (743x75; AxB scan) sampling a 7x7-mm retinal area centered at the fovea was used to obtain tomograms of the fovea. Individual B-scans at the fovea were exported and analyzed using ImageJ (Wayne Rasband, National Institute of Health) for reflectance profiles and morphologic abnormalities.Main Outcome Measures: Gross morphologic changes in OCT were characterized. Specifically, inner segment and outer segment (IS/OS) junction and cone outer segment tip (COST) disruption was noted. Using the reflectance profiles, foveal depth, thickness of the outer nuclear layer (ONL), and retinal thickness (RT) were measured.Results: A characteristic so-called punched out hyporeflective zone (HRZ) was noted in 7 of 13 patients; this was age-dependent (P = 0.001). The area of the HRZ was asymmetric with the nasal area being significantly greater than the temporal area (P = 0.002). In all patients, there was disruption of the IS/OS junction at the foveal or parafoveal regions, or both. Five of 13 patients also had a disrupted COST reflectivity. There was significant (P = 1.1x10(-6)) ONL thinning in the achromats compared with controls, which was age-dependent (P = 0.0002). Foveal maldevelopment was seen in 9 of 13 patients. The achromats also had a significantly reduced foveal depth (P = 7.7x10(-6)) and RT (Px1.46x10(-9)) compared with controls.Conclusions: A range of signs in achromatopsia are described that can be detected using UHR OCT. The IS/OS junction and COST reflectivity disruption and presence of HRZ and ONL thinning are signs of cone photoreceptor degeneration. The latter 2 are age-dependent, which suggests that achromatopsia is a progressive disorder. In addition, foveal maldevelopment is described; this represents a fetal developmental defect linked to cone photoreceptor degeneration.Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. Ophthalmology 2011;118:882-887 (C) 2011 by the American Academy of Ophthalmology.