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M. M. Ariss, Y. Qian, R. Singh, E. I. Traboulsi; Management of Lens Subluxation in Patients with Marfan Syndrome: Indications for Cataract Extraction. Invest. Ophthalmol. Vis. Sci. 2009;50(13):5571.
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© ARVO (1962-2015); The Authors (2016-present)
To review the indications and results of lens extraction in patients with Marfan Syndrome and subluxated lenses.
Retrospective chart review of patients with a definitive diagnosis of Marfan Syndrome and dislocated lenses managed at the Cleveland Clinic over a 12 year period (1996-2008). The charts of 40 patients, ages 13 months to 44 years, were reviewed. Eyes with well centered lenses and amblyopia were excluded from the study. Lens subluxation was evaluated before and after pupillary dilatation at the slit lamp under retroillumination in both primary and downgaze. The degree of lens subluxation was defined as mild (lens edge not visible through undilated pupil), moderate (lens edge visible through undilated pupil), and severe (lens subluxation progressed beyond the undilated pupil).
11 eyes were excluded from the study. Of 69 eyes, 35 were managed nonsurgically with contact lenses/spectacle correction. 29 (82.9%) lenses were graded as mildly dislocated and surgery was deferred as correction improved visual acuity better than or equal to 20/45. 4 lenses (11.4%) were graded as severely subluxated, but aphakic correction improved visual acuity better or equal to 20/40. Surgery was deferred in 2 eyes despite moderate subluxation due to an amblyopic fellow eye. 34 of 69 eyes were managed with lens extraction. The indications for surgery included 1) reduced vision and lens edge visible through undilated pupil, 18 eyes (52.9%); 2) increase in myopic astigmatism and visual acuity worse than 20/50, 9 eyes (26.5%), 3) complaints of visual distortion and fluctuating vision, 3 eyes (8.8%) 4) lens edge beyond undilated pupil and poor vision with best correction (20/60, 20/100) 2 eyes (5.9%) 5)development of cataracts, 2 eyes (5.9%). Of the surgical cases, (patient ages 6-44 years) 32 eyes were left aphakic and all achieved a visual acuity of 20/30 or better. A traumatic retinal detachment developed in one eye of a 5 year old aphakic patient. Visual acuity improved to 20/30 after RD repair. 2 patients, ages 40 and 41, had intraocular lens placement during cataract extraction of one eye. One patient had an A/C IOL and the second had a sulcus fixated PCIOL. Both had 20/25 vision and no complications.
All patients with Marfan Syndrome and mild lens subluxation can be managed with spectacle/contact lens correction and have good visual function. When increasing myopic astigmatism and signficant lens subluxation reduce vision, lensectomy results in improved visual acuity. Retinal detachment following lensectomy is uncommon, especially if eyes are left aphakic.
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