Intraocular Lens Subluxation in Marfan Syndrome

Author:

Rodrigo Bolaños-Jiménez,Paulina López-Lizárraga E,Francesc March de R,Eduardo Telich-Tarriba J,Alejandro Navas

Abstract

Purpose : Ectopia lentis (EL) is a major criteria for the diagnosis of Marfan syndrome, it may vary from an asymptomatic mild displacement to a significant subluxation that places the equator of the lens in the pupillary axis. The purpose of this work is to present the case of a patient with Marfan syndrome who received treatment for subluxation at our institution. Case Report : A 51-year-old female diagnosed with Marfan syndrome presented to the emergency department with bilateral eye redness, foreign body sensation and crusting around the eyes on awakening. She had the following history of cardiac and ophthalmologic complications, including: 1. Lens subluxation 2. High myopia 3. Aortic root dilation, 4. Mitral valve prolapse and 5. Tricuspid insufficiency. Conclusion : The ophthalmological management of Marfan patients is challenging and periodical follow-up is needed. Surgical versus conservative management is controversial, each case needs to be evaluated individually to analyze the risks and benefits of the procedures.

Publisher

Bentham Science Publishers Ltd.

Subject

Ophthalmology

Reference11 articles.

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3. Guglielmina P, Ilaria L, Lucia E , et al. Is ectopia lentis in some cases a mild phenotypic expression of Marfan syndrome? Need for a long-term follow-up. Mol Vis 2007; 13 : 2242-47.

4. Kielty CM, Davies SJ, Phillips JE, Jones CJ, Shuttleworth CA, Charles SJ. Marfan syndrome fibrillin expression and microfibrillar abnormalities in a family with predominant ocular defects. J Med Genet 1995; 32 (1) : 1-6.

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