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

Rahmani, S.; Lyon, A. T.; Fawzi, A. A.; Maumenee, I. H.; Mets, M. B.

Ophthalmic Surg Lasers Imaging Retina. 2015 Oct 16; 46(9):936-41

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.

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