[黄斑卵黄样变性]。

P Francçois, P Turut
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引用次数: 0

摘要

黄斑黄体变性是一种常染色体显性遗传疾病。这可能是先天性的。病变演变的三个阶段:一是“煎蛋”阶段。二、眼科各方面:混合性卵状、假性下视、挛缩状。在前两个阶段,淡黄色卵黄物质仅在黄斑区域延伸,尤其是向下延伸。III萎缩期。视力仍然出奇地好,尤其是在前两个阶段。然而,视觉功能的研究,特别是色觉和轮廓视野检查,发现黄斑锥的早发性,与第II期和第III期色素上皮的荧光改变有关。实际上,这些病变大多位于凹泡周围区域,尊重凹窝,这解释了良好视力的保存。在大多数情况下,眼电图被深度修改。这种影响的发病机制尚不清楚,但色素上皮和神经上皮病变的演变使我们认为卵磷脂物质位于这两层之间。
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[Vitelliform degeneration of the macula].

Macular vitelliform degeneration is an hereditary affection, with autosomal dominan transmission. It is probably congenital. Three phases in the evolution of lesions: I The "fried eggs" aspect. II Various ophthalmologic aspects: mixed egg, pseudo-hypopion, retractile form. During these first two phases, the yellowish vitelline substance only may extend in macular region, especially downards. III The atrophic phase. Visula acuity remains strangely good, especially during the first two phases. Nevertheless, the visual function study, especially colour vision and profile perimetry, elecites a precocious suffereance of macular cones, associated to fluorographic alteration of pigmentary epithelium in phases II and III. Actually, these lesions are mostly situated in perifoveolar area, respecting the foveola, which explains conservation of good vision. The electro-oculogram is deeply modified in most cases. The pathogenesis of this affection remains unexplained, but the evolution of lesions both in pigmentary epithelium and neuro-epithelium enables to think that vitellin substance lays between these two layers.

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