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Long-Term Observation of Coexistence of Posterior Polymorphous Corneal Dystrophy Resultant High Myopia and Nonkeratoconic Developing Corneal Astigmatism

机译:长期观察到后部多态性角膜营养不良导致的高度近视和非角圆锥性发展性角膜散光并存

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摘要

Posterior polymorphous corneal dystrophy (PPCD) is an extremely rare, bilateral, and inherited disorder, which affects the corneal endothelium and Descemet's membrane. Few PPCD cases in Chinese patients have been published so far. As far as we know, there are few studies which focused on the associations between PPCD and high myopia either. Here we report a rare case of coexistence of posterior polymorphous corneal dystrophy, resultant high myopia and with-the-rule developing corneal astigmatism in a young Chinese boy.A 6-year-old boy was first referred to our department 7 years ago, complaining of bilateral poor vision. Examinations of both eyes including ophthalmologic examination, cycloplegic refraction examination, confocal microscopy findings, and corneal topography were performed. Bilateral small aggregates of vesicular lesions and patchy hyperreflectivity were observed at the level of the Descemet's membrane on confocal microscopy, which is consistent with typical PPCD. Optometry and corneal topography examinations showed a resultant high myopia.Ocular examinations were performed annually to follow up with the patient in the past 7 years. The corneal lesions remained stable whereas an axial elongation and a sharp increase in both spherical and cylindrical equivalent power were observed.Close follow-ups including thorough scrutiny of the endothelium and systematic ocular ancillary examinations are essential for patients with PPCD. The pathological coexistence of PPCD and high myopia in our case is possibly due to a shared etiological pathway or genetic background. Advanced genetic analysis on similar cases is expected if more samples can be provided.
机译:后部多态性角膜营养不良(PPCD)是一种极为罕见的双侧遗传性疾病,会影响角膜内皮和Descemet膜。迄今为止,中国患者中很少有PPCD病例发表。据我们所知,很少有研究着重于PPCD和高度近视之间的关系。在这里,我们报道了一个中国男孩中并存的多态性角膜后营养不良,高度近视和规则发展的角膜散光的罕见情况.7岁的男孩在7年前首次被转介到我科双边视力差。进行了双眼检查,包括眼科检查,睫状肌麻痹验光检查,共聚焦显微镜检查结果和角膜地形图。在共焦显微镜下,在Descemet膜水平观察到双侧小水泡病变小聚集体和斑片状高反射性,这与典型的PPCD一致。验光和角膜地形图检查显示高度近视。在过去的7年中,每年对患者进行眼科检查。角膜病变保持稳定,但观察到轴向伸长以及球形和圆柱形等效屈光度均急剧增加,密切随访包括对内皮进行彻底检查和系统的眼部辅助检查对于PPCD患者至关重要。在我们的病例中,PPCD和高度近视的病理共存可能是由于共同的病因途径或遗传背景。如果可以提供更多样品,则有望对类似病例进行高级遗传分析。

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  • 年(卷),期 -1(94),23
  • 年度 -1
  • 页码 e921
  • 总页数 4
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