Distinct Characteristics of Inferonasal Fundus Autofluorescence Patterns in Stargardt Disease and Retinitis Pigmentosa

被引:28
作者
Duncker, Tobias [1 ]
Lee, Winston [1 ]
Tsang, Stephen H. [1 ,2 ]
Greenberg, Jonathan P. [1 ]
Zernant, Jana [1 ]
Allikmets, Rando [1 ,2 ]
Sparrow, Janet R. [1 ,2 ]
机构
[1] Columbia Univ, Dept Ophthalmol, New York, NY 10032 USA
[2] Columbia Univ, Dept Pathol & Cell Biol, New York, NY 10032 USA
基金
美国国家卫生研究院;
关键词
ABCA4; fundus autofluorescence; lipofuscin; melanin; optic fissure; retinal pigment epithelium; retinitis pigmentosa; scanning laser ophthalmoscope; recessive Stargardt disease; SHORT-WAVELENGTH AUTOFLUORESCENCE; MACULAR DYSTROPHY; LIPOFUSCIN; GENE; ABCR; FLAVIMACULATUS; ABNORMALITIES; VISUALIZATION; FLUORESCENCE; MUTATIONS;
D O I
10.1167/iovs.13-12895
中图分类号
R77 [眼科学];
学科分类号
100212 ;
摘要
PURPOSE. To report distinct characteristics of fundus autofluorescence (AF) patterns inferior to the optic disc in recessive Stargardt disease (STGD1) and retinitis pigmentosa (RP). METHODS. Short-wavelength (SW) and near-infrared (NIR) AF images were acquired from patients with STGD1 and RP. In SW-and NIR-AF images of STGD1 patients, gray levels (GL) on both sides of the demarcation line were measured. RESULTS. In STGD1, a demarcation line, which has been assigned to the closed optic fissure, was visible on SW-AF and NIR-AF inferior to the optic disc. In healthy subjects, this demarcation line is only visible by SW-AF. At 208 inferior to the disc center, AF levels on the nasal side were 25% (+/-11%) lower than on the temporal side in SW-AF images and 18% (+/-11%) lower in NIR-AF images. For both STGD1 and RP, the inferonasal quadrant exhibited distinct SW-and NIR-AF patterns compared with other fundus areas. Disease-related AF changes, such as flecks, appeared to respect the demarcation line as a boundary. CONCLUSIONS. Disease-related AF patterns originating in RPE in STGD1 and RP appear to respect the demarcation line in the inferonasal quadrant of the fundus as a border. The visibility of the inferonasal demarcation line by NIR-AF in STGD1 but not in healthy eyes may indicate that increased levels of RPE lipofuscin modulate the melanin-related NIR-AF signal. This feature of NIR-AF images may aid in the diagnosis of STGD1 patients.
引用
收藏
页码:6820 / 6826
页数:7
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