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马凡综合征中的视网膜疾病:来自芝加哥马凡眼联盟

Retinal Disease in Marfan Syndrome: From the Marfan Eye Consortium of Chicago.

作者信息

Rahmani Safa, Lyon Alice T, Fawzi Amani A, Maumenee Irene H, Mets Marilyn B

出版信息

Ophthalmic Surg Lasers Imaging Retina. 2015 Oct;46(9):936-41. doi: 10.3928/23258160-20151008-06.

DOI:10.3928/23258160-20151008-06
PMID:26469233
Abstract

BACKGROUND AND OBJECTIVE

To study the prevalence of peripheral retinal disease in patients with Marfan Syndrome (MFS).

PATIENTS AND METHODS

In this observational, cross-sectional case series, patients with MFS were recruited by the Marfan Eye Consortium of Chicago during the National Marfan Foundation's annual conference. Patients underwent a fully dilated exam by vitreoretinal specialists in addition to ultra-widefield fundus photography using a scanning laser ophthalmoscope (Optos 200Tx; Optos PLC, Dunfermline, Scotland, United Kingdom).

RESULTS

Clinical examination revealed posterior segment pathology in 18% of eyes with increased incidence to 70% in patients with a subluxed lens. In six out of 10 subjects in whom the clinical exam was suboptimal (young age, small pupil, and limited cooperation), the Optos provided a superior view of the peripheral retina compared to clinical exam alone.

CONCLUSION

Clinical exam of MFS patients revealed similar posterior segment pathology as noted in previous literature, with improved detection of peripheral retinal disease with the use of ultra-widefield imaging.

摘要

背景与目的

研究马凡综合征(MFS)患者周边视网膜疾病的患病率。

患者与方法

在本观察性横断面病例系列研究中,芝加哥马凡眼科联盟在国家马凡基金会年度会议期间招募了MFS患者。患者除了使用扫描激光检眼镜(Optos 200Tx;Optos PLC,英国苏格兰邓弗姆林)进行超广角眼底摄影外,还接受了玻璃体视网膜专科医生的充分散瞳检查。

结果

临床检查发现18%的眼睛存在后段病变,晶状体半脱位患者的发病率增至70%。在10名临床检查效果欠佳(年龄小、瞳孔小且配合度有限)的受试者中,有6名受试者的Optos提供的周边视网膜视野比单独临床检查更好。

结论

MFS患者的临床检查发现的后段病变与既往文献报道相似,使用超广角成像可提高周边视网膜疾病的检出率。

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