Morphological and functional analyses of adult onset vitelliform macular dystrophy
Morphological and functional analyses of adult onset vitelliform macular dystrophy
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DOI：
10.1136/bjo.87.6.758
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发表时间：
2003-06-01
影响因子：
4.1
通讯作者：
Ogata, K
中科院分区：
文献类型：
--
作者：
Saito, W;Yamamoto, S;Ogata, K
目的：评价成人卵黄样黄斑营养不良（AVMD）眼黄斑的形态和视功能。方法：采用检眼镜、扫描激光检眼镜（SLO）、光学相干断层扫描（OCT）和多焦视网膜电图（mfERGs）检查6例AVMD患者的12只眼。所有患者的黄斑病变均为双侧，包括典型的卵黄样病变（五只眼）、褪色的卵黄状改变伴视网膜色素上皮 (RPE) 萎缩（五只眼）以及黄斑周围有小斑点的正常黄斑中心凹（两只眼）。 结果：在所有病例中，SLO 在整个后视网膜的深部视网膜中均显示出小的异常亮点。 OCT 显示黄斑下黄色卵黄样病变患者的 RPE 和脉络膜毛细血管水平存在高反射梭形增厚层。在伴有 RPE 萎缩的黄斑病变的视网膜层下方观察到界限清楚、光学清晰的空间。 mfERGs不仅在黄斑区显着减少，而且在mfERGs的最外环（20-30度）也显着减少。 结论：OCT可以检测到先前卵黄样病变组织病理学研究中报道的RPE或上皮下层内积累的黄斑下物质。在伴有 RPE 萎缩的黄斑病变中，物质可能已经消失，留下视网膜下或上皮下光学清晰空间。这些 SLO 和 mfERG 观察结果表明，形态和功能异常可能不仅仅局限于黄斑区，而是可能存在于患有 AVMD 的眼睛的整个后极。
Aim: To evaluate the morphology and visual function of the macula in eyes with adult onset vitelliform macular dystrophy ( AVMD).Methods: 12 eyes of six patients with AVMD were examined by ophthalmoscopy, scanning laser ophthalmoscopy (SLO), optical coherence tomography (OCT), and multifocal electroretinography ( mfERGs). The macular lesions were bilateral in all patients and varied from the typical vitelliform ( five eyes), faded vitelliform changes with retinal pigment epithelium ( RPE) atrophy ( five eyes), and a normal fovea associated with small flecks around the macula ( two eyes).Results: SLO demonstrated small abnormal bright spots in the deep retina throughout the posterior retina in all cases. OCT showed a highly reflective fusiform thickened layer at the level of the RPE and choriocapillaris in patients with a submacular yellow vitelliform lesion. A well circumscribed, optically clear space was observed beneath the retinal layer in the macular lesions with RPE atrophy. The mfERGs were significantly reduced not only in the macular area but also in the outermost ring (20 - 30degrees) of the mfERGs.Conclusions: The submacular materials that accumulate within the RPE or subepithelial layers reported in previous histopathological studies of vitelliform lesions can be detected by OCT. In the macular lesions with RPE atrophy, the material may have disappeared leaving a subretinal or subepithelial optical clear space. These SLO and mfERG observations suggest that the morphological and functional abnormalities may not be localised just in the macular area but may be present throughout the posterior pole in eyes with AVMD.