患有马方综合征的儿童的角膜特征,有或没有ectopia lentis
Marisa O'Brien1, Lawrence Tychsen1,2,3
1Department of Ophthalmology and Visual Sciences St. Louis Children's Hospital at Washington University Medical Center St. Louis Missouri USA.
Pediatric investigation
|June 20, 2025
概括
与没有EL的儿童相比,患有马凡综合征 (MFS) 和角膜外垂 (EL) 的儿童在角膜结构和轴长度上有显著差异. 这些发现表明,在患有EL的MFS患者中,纤维素缺陷更严重.
科学领域:
- 眼科医生 眼科 眼科
- 遗传学 是一个遗传学.
- 儿科 儿科 儿科
背景情况:
- 马凡综合征 (MFS) 是一种影响结缔组织的遗传疾病,通常导致眼部异常.
- 在MFS的近视与透镜外置,异常的角膜曲率和轴长度增加有关.
- 有限的研究存在于MFS儿童的眼睛参数与没有ectopia lentis (EL).
研究的目的:
- 纵向评估和比较患有马凡综合征的儿童的眼部发现,通过存在或不存在ectopia lentis进行分层.
- 为了研究这些儿科MFS队列中的折射误差,角膜结构和轴长度的进展.
主要方法:
- 一个前性,比较的案例研究,涉及24名患有MFS的儿童 (48只眼睛).
- 参与者被分为两组:16名EL需要手术,8名没有EL (phakic).
- 包括视敏度,折射误差,中角膜厚度 (CCT),内皮细胞密度 (ECD),角膜曲率和轴长度在内的眼睛参数在平均后续3.1年内被监测.
主要成果:
- 与没有EL的儿童相比,MFS患有EL的儿童在基线呈现出更高的近视,更大的角膜圆柱体和更厚的CCT.
- 随着时间的推移,EL患者呈现出渐进的CCT增加,ECD减少,角膜平整,角膜圆柱体缩小和轴长增加.
- 没有EL的儿童表现出CCT的较小增加和较轻的角膜平,ECD或轴长度没有显著变化.
结论:
- 马方综合征中Ectopia lentis与角膜结构和轴长度的明显变化有关.
- 患有EL的MFS儿童表现出更厚的角膜,更高的角膜形,减少了内皮细胞密度和更长的轴长度.
- 这些眼睛的差异表明,在MFS患者中,EL患者的底层纤维素缺陷更严重,不仅影响了透镜,还影响了角膜和质完整性.
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