von Rückmann A, Fitzke F W, Bird A C
Institute of Ophthalmology, Moorfields Eye Hospital, London, England.
Arch Ophthalmol. 1997 May;115(5):609-15. doi: 10.1001/archopht.1997.01100150611006.
To document the deviation from normal of fundus autofluorescence in patients with inherited macular dystrophies.
The intensity and spatial distribution of fundus autofluorescence was documented in 118 patients with inherited macular dystrophies by means of a confocal laser scanning ophthalmoscope, and the images were compared with the fundus appearance and fluorescein angiograms.
Background autofluorescence appears to be elevated in all forms of macular dystrophies examined. The pale deposits at the level of the retinal pigment epithelium in disorders such as Best disease, adult vitelliform macular dystrophy, and fundus flavimaculatus were consistently associated with higher levels of autofluorescence than the background signal. There was no strong correlation between the intensity of autofluorescence and the fluorescein angiographic sign of a dark choroid. Increased levels of autofluorescence were present in a subject with a mutation known to cause macular dystrophy but in whom there were no manifest ophthalmoscopic or functional abnormalities.
All dystrophies examined have in common accumulation of autofluorescent material in the retinal pigment epithelium to a greater degree than that seen with age. The abnormal high background autofluorescence associated with inherited macular dystrophies confirms the impression derived from histological studies that these disorders affect the entire retinal pigment epithelium. The lack of correlation between autofluorescence and the presence of a dark choroid implies that there may be different fluorophores in different disorders. The pale deposits at the level of the retinal pigment epithelium-Bruch membrane seen in macular dystrophies have similar autofluorescence characteristics. This technique may be useful in detecting the abnormal phenotype in early disease.
记录遗传性黄斑营养不良患者眼底自发荧光与正常情况的偏差。
使用共焦激光扫描检眼镜记录118例遗传性黄斑营养不良患者眼底自发荧光的强度和空间分布，并将图像与眼底外观及荧光素血管造影进行比较。
在所检查的所有黄斑营养不良形式中，背景自发荧光似乎均升高。在诸如Best病、成人卵黄样黄斑营养不良和黄斑黄素沉着症等疾病中，视网膜色素上皮水平的苍白沉积物始终与高于背景信号的自发荧光水平相关。自发荧光强度与脉络膜暗荧光素血管造影征象之间无强相关性。在一名已知有导致黄斑营养不良的突变但无明显眼底镜或功能异常的受试者中，存在自发荧光水平升高的情况。
所有检查的营养不良均具有共同特征，即视网膜色素上皮中自发荧光物质的积累程度高于随年龄增长所见。与遗传性黄斑营养不良相关的异常高背景自发荧光证实了组织学研究得出的印象，即这些疾病会影响整个视网膜色素上皮。自发荧光与脉络膜暗斑之间缺乏相关性意味着不同疾病中可能存在不同的荧光团。在黄斑营养不良中所见的视网膜色素上皮 - Bruch膜水平的苍白沉积物具有相似的自发荧光特征。该技术可能有助于在疾病早期检测异常表型。
