sclerocornea

硬化角膜
  • 文章类型: Journal Article
    角膜平面(CP)是一种罕见的眼部疾病,存在两种不同的临床和遗传形式:较温和,常染色体显性I型和更严重的,常染色体隐性遗传II型。这种情况在芬兰语中更常见,沙特,捷克家庭我们报告了来自近亲婚姻的三个兄弟,他们抱怨视力不同程度地下降。他们三个都是蓝色的,厚,和前房深度浅的朦胧角膜。CPII型的其他特征在两个哥哥中可见,包括弧菌类脂,界限不清,和一个舒适的斜视。通过眼镜矫正屈光不正和详细的咨询以及随访以寻找进行性并发症来管理他们。管理主要围绕光学或手术纠正发育异常。适当的遗传咨询和定期随访以寻找和管理并发症。有,然而,这些患者可以考虑的新疗法包括角膜移植或角膜基质干细胞疗法。
    Cornea plana (CP) is a rare ocular condition existing in two distinct clinical and hereditary forms: a milder, autosomal dominant type I and a more severe, autosomal recessive type II. The condition is more commonly found in Finnish, Saudi, and Czech families. We report three brothers from a consanguineous marriage that presented with complaints of decreased vision of varying degrees. All three of them have blue, thick, and hazy corneas with shallow anterior chamber depths. The additional features of CP type II were seen in the older two brothers including arcus lipoids, ill-demarcated limbus, and an accommodative squint. They were managed by the correction of refractive errors through spectacles and detailed counseling with follow-up visits to look for progressive complications. The management is mainly centered around optically or surgically correcting the developmental anomalies. This is complimented with proper genetic counseling and regular follow-up visits to look for and manage complications. There are, however, novel therapies that can be considered in these patients including corneal transplants or corneal stromal stem cellular therapies.
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