Anterior scleral thickness in Marfan syndrome: A quantitative analysis

IF 3 3区医学 Q1 OPHTHALMOLOGY Acta Ophthalmologica Pub Date : 2024-05-21 DOI:10.1111/aos.16721

Lien Alluyn, Laure Dequeker, Siska Dhaese, Alejandra Consejo, Julie De Zaeytijd, Bart P. Leroy, Julie De Backer, Elke O. Kreps

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Abstract

Purpose

To investigate the anterior scleral thickness (AST) in patients with Marfan syndrome (MFS).

Methods

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.

Results

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.

Conclusion

Based on anterior segment OCT measurements, AST of patients with MFS is significantly lower compared to matched controls.

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马凡氏综合征的前巩膜厚度：定量分析

目的：研究马凡氏综合征（MFS）患者的前巩膜厚度（AST）：根特市根特大学医院眼科开展了一项前瞻性横断面研究，研究对象包括经临床基因确诊的马凡氏综合征患者以及年龄、性别和轴长匹配的对照组。排除已知角膜、结膜或巩膜病变以及眼部手术史（包括玻璃体旁切除术）、近期使用隐形眼镜或高度散光的受试者。受试者接受了非环视自动屈光检查、基于 Scheimpflug 的角膜断层扫描、轴向长度测量和光谱域光学相干断层扫描（OCT）。在距巩膜距 1 毫米（AST1）、2 毫米（AST2）和 3 毫米（AST3）处，分别从时间和鼻侧对 AST 进行人工测量：本研究共纳入 56 名受试者（MFS 组 28 名，对照组 28 名）。与配对对照组相比，MFS 患者的 AST 明显降低，无论是整体还是鼻部和颞部的每个分析测量点都是如此（p 结论：MFS 患者的 AST 明显低于配对对照组：根据前段 OCT 测量结果，与匹配的对照组相比，MFS 患者的 AST 明显降低。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Acta Ophthalmologica 医学-眼科学

CiteScore

7.60

自引率

5.90%

发文量

433

审稿时长

6 months

期刊介绍： Acta Ophthalmologica is published on behalf of the Acta Ophthalmologica Scandinavica Foundation and is the official scientific publication of the following societies: The Danish Ophthalmological Society, The Finnish Ophthalmological Society, The Icelandic Ophthalmological Society, The Norwegian Ophthalmological Society and The Swedish Ophthalmological Society, and also the European Association for Vision and Eye Research (EVER). Acta Ophthalmologica publishes clinical and experimental original articles, reviews, editorials, educational photo essays (Diagnosis and Therapy in Ophthalmology), case reports and case series, letters to the editor and doctoral theses.