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[卵黄样黄斑变性的各个阶段]

[The various stages of vitelliform macular degeneration].

作者信息

Lisch W

出版信息

Klin Monbl Augenheilkd. 1980 Feb;176(2):214-21. doi: 10.1055/s-2008-1057430.

Abstract

Hereditary autosomal dominant vitelliform macular degeneration and Best's macular degeneration are synonyma. The different namens for this disease rise from its varying clinical manifestation. The age at which it becomes manifest also varies. Our findings in two families with this hereditary foveal disease confirm that the macular changes are polymorphous. Nine different characteristic states of vitelliform macular degeneration can be distinguished. The vitelliform stage may represent a previtelliform or a resorptive stage. The differential diagnosis between vitelliform disc and vitelliform cyst can only be made unequivocally by fluorescein angiography. If the vitelliform cyst is scarred directly the scar is sharply delineated. A vitelliform stage or a pseudohypopyon stage may develop, especially form larger vitelliform cysts. In such cases the final stage may be a poorly delineated foveal scar. Occassionally, a mixed picture of two stages is observed, e.g., of cystic and scarred states. Exact knowledge of all stages of the disease is of great interest with resepct to differential diagnosis.

摘要

遗传性常染色体显性卵黄样黄斑变性和贝斯特黄斑变性是同义词。这种疾病的不同名称源于其不同的临床表现。其发病年龄也各不相同。我们对两个患有这种遗传性黄斑疾病的家族的研究结果证实,黄斑病变是多形性的。卵黄样黄斑变性可区分出九种不同的特征状态。卵黄样阶段可能代表前卵黄样或吸收阶段。卵黄样盘和卵黄样囊肿之间的鉴别诊断只能通过荧光素血管造影明确做出。如果卵黄样囊肿直接形成瘢痕,瘢痕边界清晰。可能会出现卵黄样阶段或假前房积脓阶段,尤其是较大的卵黄样囊肿。在这种情况下,最终阶段可能是边界不清的黄斑瘢痕。偶尔会观察到两个阶段的混合表现,例如囊肿和瘢痕状态。准确了解疾病的所有阶段对于鉴别诊断非常重要。

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