Department of Ophthalmology, Ghent University Hospital, Ghent, Belgium.
Department of Ophthalmology, AZ Groeninge, Kortrijk, Belgium.
Acta Ophthalmol. 2024 Nov;102(7):e1050-e1056. doi: 10.1111/aos.16721. Epub 2024 May 21.
To investigate the anterior scleral thickness (AST) in patients with Marfan syndrome (MFS).
A prospective, cross-sectional study was conducted at the Department of Ophthalmology, Ghent University Hospital, Ghent, including patients with a genetically confirmed clinical diagnosis of MFS and age-, gender- and axial length-matched controls. Subjects with known corneal, conjunctival or scleral pathology and a history of ocular surgery, including pars plana vitrectomy, recent contact lens use or high-grade astigmatism were excluded. Subjects underwent non-cycloplegic autorefraction, Scheimpflug-based corneal tomography, axial length measurement and spectral-domain optical coherence tomography (OCT). AST was manually measured at 1 mm (AST1), 2 mm (AST2) and 3 mm (AST3) from the scleral spur, temporally and nasally.
A total of 56 subjects (28 subjects in the MFS group and 28 matched subjects in the control group) were included in this study. In patients with MFS, AST was significantly reduced compared to matched controls, both overall and at every analysed measuring point in the nasal and temporal areas (p < 0.001). Central corneal thickness (CCT) and mean keratometry (Kmean) values were significantly lower in patients with MFS (p < 0.05). A positive correlation was found between nasal AST and CCT in patients with MFS. No correlation was found between AST and Kmean or between AST and axial length. In patients with MFS with ectopia lentis, compared to those without, temporal AST3 was significantly lower (p < 0.05). AST was significantly lower in patients with MFS harbouring a variant predicted to cause haploinsufficiency compared to those with a variant expected to lead to a dominant negative effect for both nasal and temporal measurements.
Based on anterior segment OCT measurements, AST of patients with MFS is significantly lower compared to matched controls.
研究马凡综合征（MFS）患者的前巩膜厚度（AST）。
在根特大学医院眼科进行了一项前瞻性、横断面研究，纳入了经基因证实的临床诊断为 MFS 的患者和年龄、性别和眼轴长度匹配的对照组。排除已知角膜、结膜或巩膜疾病以及眼部手术史的患者，包括睫状体平坦部玻璃体切除术、近期隐形眼镜使用或高度散光。患者接受非睫状肌麻痹自动验光、基于 Scheimpflug 的角膜断层扫描、眼轴长度测量和光谱域光相干断层扫描（OCT）。在巩膜突 1mm（AST1）、2mm（AST2）和 3mm（AST3）处手动测量巩膜厚度，分别在颞侧和鼻侧进行测量。
本研究共纳入 56 名受试者（MFS 组 28 名，对照组 28 名）。与对照组相比，MFS 患者的 AST 整体以及在鼻侧和颞侧的每个分析测量点均显著降低（p<0.001）。MFS 患者的中央角膜厚度（CCT）和平均角膜曲率（Kmean）值显著较低（p<0.05）。MFS 患者的鼻侧 AST 与 CCT 呈正相关。AST 与 Kmean 或 AST 与眼轴之间无相关性。与无晶状体异位的 MFS 患者相比，有晶状体异位的 MFS 患者的颞侧 AST3 显著降低（p<0.05）。与预计导致显性负效应的变异相比，预测导致杂合子功能不全的变异的 MFS 患者的鼻侧和颞侧 AST 均显著降低。
基于眼前节 OCT 测量，MFS 患者的 AST 明显低于对照组。
