Management Strategies of Ocular Abnormalities in Patients with Marfan Syndrome: Current Perspective

Journal of Ophthalmic & Vision Research
Hamed EsfandiariMarilyn B Mets

Abstract

Marfan syndrome is an autosomal dominant genetic connective tissue disorder that results from mutations in the fibrillin-1 gene located on chromosome band 15q15-21. Fibrillin, a glycoprotein, is widely expressed throughout the body and contributes to the elasticity and force-bearing capacity of connective tissue. In the eye, fibrillin is a key constituent of the ciliary zonules, which suspend the crystalline lens in place. The zonular defect leads to ectopia lentis, which is a hallmark of Marfan ocular abnormalities and occurs in 60% to 80% of cases. Other less common ocular features of Marfan syndrome are increased axial length, astigmatism, and flat cornea. Visual function in Marfan syndrome could be affected in several ways: ectopia lentis, refractive error, amblyopia, retinal detachment, cataract, and glaucoma. Management of a subluxated lens starts with the correction of refractive error with eyeglasses in mild cases. In more severe cases, especially when the lens bisects the pupil, complete correction of refractive error is impossible without removing the subluxated lens. The best method for visual rehabilitation after lens extraction is still debated. Aphakic Artisan lens implantation at the time of subluxated lens remov...Continue Reading

References

Dec 1, 1996·Journal of Vascular Nursing : Official Publication of the Society for Peripheral Vascular Nursing·D StriderI Kron
Oct 15, 1998·British Journal of Plastic Surgery·M SaleemM Babu Manohar
Dec 18, 2003·The American Journal of Medicine·John D Brunzell, Amir F Ayyobi
Oct 31, 2009·American Journal of Ophthalmology·Jae Hoon KimJae Chan Kim
Jun 12, 2010·Bulletin of the World Health Organization·Robert Fryatt, Anne Mills
May 11, 2012·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·R B HopkinsL Thabane
Jul 5, 2013·Journal of Human Hypertension·D KilicH Evrengul
Jan 24, 2015·The British Journal of Psychiatry : the Journal of Mental Science·Tobias GerhardMark Olfson
May 21, 2016·BMJ Case Reports·Kiranmaye TuragaSubhadra Jalali

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Citations

Sep 5, 2020·Clinical Case Reports·Omid DaneshjooFederica Sangiuolo
Jun 9, 2020·Journal of Cataract and Refractive Surgery·Sarah H Van Tassell
Jan 12, 2021·Clinical & Experimental Ophthalmology·Michael J Ang, Natalie A Afshari
Jan 25, 2021·Experimental Eye Research·Ruchi ShahAlexander V Ljubimov
Jan 18, 2022·Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Für Klinische Und Experimentelle Ophthalmologie·Muhammed Nurullah BulutŞaban Şimşek

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