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多灶性卵黄样黄斑营养不良的高分辨率光谱域光学相干断层扫描结果

High-resolution spectral domain optical coherence tomography findings in multifocal vitelliform macular dystrophy.

作者信息

Querques Giuseppe, Regenbogen Michael, Soubrane Gisele, Souied Eric H

机构信息

Department of Ophthalmology, University of Paris XI, Creteil, France.

出版信息

Surv Ophthalmol. 2009 Mar-Apr;54(2):311-6. doi: 10.1016/j.survophthal.2008.12.010.

Abstract

We describe the abnormalities seen in the mid periphery and posterior pole of two patients with multifocal vitelliform macular dystrophy as evaluated by high-definition spectral domain optical coherence tomography (HD-OCT). In patient 1, HD-OCT scans revealed, in the central area, a thicker and more reflective layer compared with the normal macula, located between the retinal pigment epithelium and the interface of the inner segment/outer segment, corresponding to the Verhoeff's membrane. Moreover, HD-OCT macular scans, as well as C-scans, revealed a slight hyper-reflective lesion just above an area of reduced reflectivity between the photoreceptor layer (interface of the inner segment and outer segment) and the Verhoeff's membrane. In patient 2, on HD-OCT macular scans, the layer corresponding to the interface of inner segment and outer segment of the photoreceptor, and the Verhoeff's membrane, appeared disrupted, whereas the retinal pigment epithelium layer appeared preserved. On the other hand, in both patient 1 and 2, the clinically evident vitelliform lesions outside the macular area appeared on HD-OCT scans either as small focal hyper-reflective lesions at the level of the retinal pigment epithelium/photoreceptor complex, either as a more pronounced diffuse thickening of the retinal pigment epithelium/photoreceptor complex, facing the deposition of lipofuscin reported on the histopathologic examination. These new findings would help in a further understanding of multifocal vitelliform macular dystrophy.

摘要

我们描述了通过高分辨率光谱域光学相干断层扫描(HD-OCT)评估的两名多灶性卵黄样黄斑营养不良患者中周部和后极部的异常情况。在患者1中,HD-OCT扫描显示,在中央区域,与正常黄斑相比,位于视网膜色素上皮和内节/外节界面之间有一层更厚且反射更强的层,对应于韦尔霍夫膜。此外,HD-OCT黄斑扫描以及C扫描显示,在光感受器层(内节和外节界面)与韦尔霍夫膜之间反射率降低区域上方有一个轻微的高反射性病变。在患者2中,HD-OCT黄斑扫描显示,对应于光感受器内节和外节界面以及韦尔霍夫膜的层出现中断,而视网膜色素上皮层似乎保存完好。另一方面,在患者1和患者2中,黄斑区外临床上明显的卵黄样病变在HD-OCT扫描中,要么表现为视网膜色素上皮/光感受器复合体水平的小灶性高反射性病变,要么表现为视网膜色素上皮/光感受器复合体更明显的弥漫性增厚,这与组织病理学检查中报告的脂褐素沉积情况相符。这些新发现将有助于进一步了解多灶性卵黄样黄斑营养不良。

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