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在Marfan综合征与ectopia lentis中的生物识别和角膜特征

L Izquierdo¹, I Gomez², C Moctezuma²

¹Research Department, Oftalmosalud Institute of Eyes, Av. Javier Prado Este 1142, San Isidro, Lima 27, Peru; Universidad Nacional Mayor de San Marcos, Lima, Peru.

Journal francais d'ophtalmologie

|February 21, 2024

概括

马凡综合征 (MFS) 带有外角膜 (EL) 显示轴长度增加,角膜更平坦,更厚. 在这些患者中,角膜生物力学和后部升高保持正常.

科学领域:

眼科医生眼科眼科
遗传学遗传学是一个
角质科学角质科学

背景情况:

马凡综合征 (MFS) 是一种影响结缔组织的遗传性疾病.
乳眼 (EL) 是MFS的一个常见的眼部表现,涉及透镜位移.
了解MFS与EL中的角膜特征对于管理眼部并发症至关重要.

研究的目的:

为了描述被诊断为马方综合征和乳头外皮症的患者的生物识别和角膜特征.
提供有关角膜形状,厚度,生物力学和这一特定患者队列中异常的详细数据.

主要方法:

进行了一项观察性,描述性,前性研究.
进行了Scheimpflug分析 (Pentacam),轴长度 (AL) 测量 (IOL Master 700),内皮细胞计数 (ECC) 和角膜生物力学评估 (ORA,Corvis).
分析了来自MFS和EL的23名患者的44只眼睛的数据.

主要成果:

透镜外置最常见的是超鼻腔 (40.9%).
平均轴长为25.63±3.65mm;角膜更平 (平均角质测量为40.22±1.76 D) 和更厚,有更高的正则 (1.68±0.83 D).
角膜生物力学指数 (CBI,TBI),后部升高和密度计都在正常范围内.

结论:

患有马凡氏综合征和外皮斜视症的患者表现出轴长度增加和角膜形态变化 (更平坦,更厚,更高的纹).
在这个人群中,角膜的生物力学特性和后部升高似乎不受影响.
这些发现有助于更好地了解患有EL的MFS的眼睛表型.

关键词:

轴长度轴长度轴长度生物机械的角质.视角膜视角膜是一个角质生物力学角质生物力学一个角形的角形.轴的长度轴的长度马芬综合征是什么意思马芬综合征是什么意思马尔芬综合征是什么意思

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