Correlations Among Near-Infrared and Short-Wavelength Autofluorescence and Spectral-Domain Optical Coherence Tomography in Recessive Stargardt Disease
Correlations Among Near-Infrared and Short-Wavelength Autofluorescence and Spectral-Domain Optical Coherence Tomography in Recessive Stargardt Disease
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DOI：
10.1167/iovs.14-14848
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发表时间：
2014-12-01
影响因子：
4.4
通讯作者：
Sparrow, Janet R.
中科院分区：
文献类型：
--
作者：
Duncker, Tobias;Marsiglia, Marcela;Sparrow, Janet R.
目的。短波（SW）眼底自身荧光（AF）被认为来源于视网膜色素上皮（RPE）中的脂褐素，而近红外（NIR） AF被认为来源于黑色素。在隐性Stargardt病（STGD1）患者中，我们将SW-AF和NIR-AF与光谱域光学相干断层扫描（SD-OCT）获得的结构信息相关联。前瞻性研究了24例STGD1患者（45眼，年龄8 - 61岁）携带确认的疾病相关ABCA4突变。获得短波自动对焦、NIR-AF和SD-OCT图像。根据中心病变的特征和眼底改变的程度确定了五种表型。NIR-AF减少的中心区域通常大于SW-AF减少的区域，NIR-AF减少的区域通常近似于SD-OCT识别的椭球区（EZ）损失区域（组1；r, 0.93, P < 0.0001）。在中心病变伴NIR-AF和SW-AF增加的重叠旁凹环的患者（3组），EZ损失程度与NIR-AF环内径密切相关（r, 0.89, P < 0.0001），且NIR-AF环外缘偏心度大于SW-AF环偏心度。与SWAF相比，NIR-AF图像更完整地描绘了病变区域。在大多数情况下，仅在NIR-AF减少或缺失的位置观察到EZ损失，表明RPE细胞萎缩发生在光感受器细胞变性之前。由于SW-AF通常在EZ破坏的中心区域增加，退化的光感受器细胞可能会加速产生脂褐素。考虑到超nir - af与SW-AF增加的潜在机制。
PURPOSE. Short-wavelength (SW) fundus autofluorescence (AF) is considered to originate from lipofuscin in retinal pigment epithelium (RPE) and near-infrared (NIR) AF from melanin. In patients with recessive Stargardt disease (STGD1), we correlated SW-AF and NIR-AF with structural information obtained by spectral-domain optical coherence tomography (SD-OCT).METHODS. Twenty-four STGD1 patients (45 eyes; age 8 to 61 years) carrying confirmed disease-associated ABCA4 mutations were studied prospectively. Short-wavelength AF, NIR-AF, and SD-OCT images were acquired.RESULTS. Five phenotypes were identified according to features of the central lesion and extent of fundus change. Central zones of reduced NIR-AF were typically larger than areas of diminished SW-AF and reduced NIR-AF usually approximated areas of ellipsoid zone (EZ) loss identified by SD-OCT (group 1; r, 0.93, P < 0.0001). In patients having a central lesion with overlapping parafoveal rings of increased NIR-AF and SW-AF (group 3), the extent of EZ loss was strongly correlated with the inner diameter of the NIR-AF ring (r, 0.89, P < 0.0001) and the eccentricity of the outer border of the NIR-AF ring was greater than that of the SW-AF ring.CONCLUSIONS. Lesion areas were more completely delineated in NIR-AF images than with SWAF. In most cases, EZ loss was observed only at locations where NIR-AF was reduced or absent, indicating that RPE cell atrophy occurs in advance of photoreceptor cell degeneration. Because SW-AF was often increased within the central area of EZ disruption, degenerating photoreceptor cells may produce lipofuscin at accelerated levels. Consideration is given to mechanisms underlying hyper-NIR-AF in conjunction with increased SW-AF.