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富克斯角膜营养不良。角膜水肿变异的临床病理研究。

Fuchs' corneal dystrophy. A clinicopathologic study of the variation in corneal edema.

作者信息

Rodrigues M M, Krachmer J H, Hackett J, Gaskins R, Halkias A

出版信息

Ophthalmology. 1986 Jun;93(6):789-96.

PMID:3526227
Abstract

Corneal buttons from six patients with Fuchs' dystrophy had varying degrees of clinical edema measured in most cases by preoperative optical or ultrasonic pachymetry. These were sectioned in the operating room so that histologic correlations could be made. Histologically, marked thickening of Descemet's membrane and abnormal corneal endothelium corresponded to areas of severe clinical edema and were usually located in the central and paracentral regions. Descemet's membrane displayed multiple prominent guttata of varying size and shape, either facing the anterior chamber, or buried within multilaminar Descemet's membrane. In some corneas, aggregates of 10 nm fibrils were seen at the edges of guttata, corresponding to areas that stained for oxytalan fibrils. The endothelium was attenuated underlying the guttata. Clinical edema was not present unless accompanied by marked thickening of Descemet's membrane with multiple guttata and attenuation of corneal endothelium. The peripheral cornea was relatively clear clinically and showed minimal histologic changes.

摘要

来自6例Fuchs角膜内皮营养不良患者的角膜片,多数病例通过术前光学或超声角膜测厚法测量,显示出不同程度的临床水肿。这些角膜片在手术室进行切片,以便进行组织学对照。组织学上,Descemet膜明显增厚和角膜内皮异常与严重临床水肿区域相对应,且通常位于中央和旁中央区域。Descemet膜呈现出多个大小和形状各异的明显滴状赘生物,有的面向前房,有的则埋于多层Descemet膜内。在一些角膜中,在滴状赘生物边缘可见10纳米的原纤维聚集体,对应于用弹力纤维染色的区域。滴状赘生物下方的内皮变薄。除非伴有Descemet膜明显增厚、多个滴状赘生物和角膜内皮变薄,否则不会出现临床水肿。周边角膜临床相对清晰,组织学变化 minimal(原文此处可能有误,推测可能是minimal,意为“最小的、极少的”)。

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