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      Intraocular Lens Subluxation in Marfan Syndrome

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          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.

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          Marfan syndrome: clinical diagnosis and management.

          John Dean (2007)
          Marfan syndrome is a multisystem connective tissue disorder usually associated with mutation in fibrillin, and occasionally with mutation in TGFBR1 or 2. The clinical diagnosis is made using the Ghent nosology, which will unequivocally diagnose or exclude Marfan syndrome in 86% of cases. Use of a care pathway can help implementation of the nosology in the clinic. The penetrance of some features is age dependent, so the nosology must be used with caution in children. Molecular testing may be helpful in this context. The nosology cannot be used in families with isolated aortic dissection, or with related conditions such as Loeys-Dietz syndrome, although it may help identify families for further diagnostic evaluation because they do not fulfill the nosology, despite a history of aneurysm. Prophylactic medical (eg beta-blockade) and surgical intervention is important in reducing the cardiovascular complications of Marfan syndrome. Musculoskeletal symptoms are common, although the pathophysiology is less clear--for example, the correlation between dural ectasia and back pain is uncertain. Symptoms in other systems require specialist review such as ophthalmology assessment of refractive errors and ectopia lentis. Pregnancy is a time of increased cardiovascular risk for women with Marfan syndrome, particularly if the aortic root exceeds 4 cm at the start of pregnancy. High-intensity static exercise should be discouraged although low-moderate intensity dynamic exercise may be beneficial. The diagnosis and management of Marfan syndrome requires a multidisciplinary team approach, in view of its multisystem effects and phenotypic variability.
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            Outcomes of iris-claw anterior chamber versus iris-fixated foldable intraocular lens in subluxated lens secondary to Marfan syndrome.

            Denise Hirashima, Eduardo Soriano, Rodrigo Meirelles … (2010)
            To compare the outcome of phacoemulsification using 2 different iris-fixation techniques for intraocular lens (IOL) replacement, a foldable posterior chamber IOL (PCIOL; AcrySof MA60AC, Alcon Laboratories Inc, Fort Worth, TX) and an iris-claw anterior chamber IOL (ACIOL; Artisan, Ophtec BV), for treatment of subluxated lenses in patients with Marfan syndrome (MFS).
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              Hereditary subluxated lenses: visual performances and long-term follow-up after surgery.

              Irene Anteby, Maram Isaac, David BenEzra (2003)
              To evaluate the visual functions of children with hereditary dislocated lenses after within-the-bag lensectomy surgery and to report the results of 18 eyes with a follow-up interval of 11 to 19 years.
              Article 0 comments Cited 12 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Open Ophthalmol J
                Journal ID (iso-abbrev): Open Ophthalmol J
                Journal ID (publisher-id): TOOPHTJ
                Title: The Open Ophthalmology Journal
                Publisher: Bentham Open
                ISSN (Electronic): 1874-3641
                Publication date (Electronic): 19 September 2014
                Publication date Collection: 2014
                Volume: 8
                Pages: 48-50
                Affiliations
                [1 ]Department of Opthalmology, Regional Hospital Adolfo López Mateos, ISSSTE, Mexico City, Mexico
                [2 ]Research Unit, Institute of Ophthalmology, Fundación Conde de Valenciana, Mexico City, Mexico
                [3 ]Department of Anterior Segment, Institute of Ophthalmology, Fundación Conde de Valenciana, Mexico City, Mexico
                [4 ]Department of Vitreoretinal Surgery, Institute of Ophthalmology, Fundación Conde de Valenciana, Mexico City, Mexico
                [5 ]Department of Anterior Segment, Dr. Luis Sánchez Bulnes Hospital, Asociación Para Evitar la Ceguera en México, Mexico City, Mexico
                [6 ]National Institute of Cardiology "Ignacio Chávez", Mexico City, Mexico
                [7 ]Department of Cornea and Refractive Surgery Institute of Ophthalmology, Fundación Conde de Valenciana, Mexico City, Mexico
                Author notes
                [* ]Address correspondence to this author at the Department of Opthalmology, Regional Hospital Adolfo López Mateos, ISSSTE, Mexico City, Mexico; Fax: 525552345685; E-mail: rodrigoboji88@ 123456gmail.com
                Article
                Publisher ID: TOOPHTJ-8-48
                DOI: 10.2174/1874364101408010048
                PMC ID: 4181168
                PubMed ID: 25279020
                SO-VID: 80592434-c44b-406a-9eeb-74bd18655785
                Copyright © © Rodrigo et al.; Licensee Bentham Open.
                License:

                This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

                History
                Date received : 18 July 2014
                Date revision received : 21 July 2014
                Date accepted : 21 July 2014
                Categories
                Subject: Article

                ScienceOpen disciplines: Ophthalmology & Optometry
                Keywords: capsular tension ring,intraocular lens subluxation,zonular dyalisis,zonular instability.
                Data availability:
                ScienceOpen disciplines: Ophthalmology & Optometry
                Keywords: capsular tension ring, intraocular lens subluxation, zonular dyalisis, zonular instability.

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