Marfan syndrome is an autosomal dominant genetic connective tissue disorder that results from mutations in the fibrillin-1 gene located on chromosome band 15q 15–21. Fibrillin, a glycoprotein, is widely expressed throughout the body and contribute to 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 that is 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 subluxated lens starts with correction of refractive error with eyeglasses in mild cases. In more severe cases, especially when lens bisects the pupil, complete correction of refractive error is impossible without removing subluxated lens. The best method for visual rehabilitation after lens extraction is still debated. Aphakic Artisan implantation at the time of subluxated lens removal results in good visual outcomes with acceptable safety profile. Studies with longer term follow up and larger sample populations are needed to evaluate the safety of this procedure in patients with Marfan syndrome.
single-piece IOL exchange with 3-piece IOL dramatically decreases pigment release and reduces IOP. Those with advanced age and higher IOP are less likely to respond to IOL exchange and may need glaucoma surgery to control high intraocular pressure.
Artisan IOL implantation after lens extraction appears to be an attractive alternative for optical correction in cases of Marfan syndrome with ectopia lentis. It confers a significant improvement in visual acuity with reasonable risk profile.
PURPOSE: To evaluate the results of lamellar crescentic resection in pellucid marginal corneal degeneration.
METHODS: Patients with pellucid marginal corneal degeneration who underwent lamellar crescentic resection from 1995 to 2000 at Labbafinejad Medical Center were assessed. Diagnosis was based on clinical findings of slit-lamp microscopy and confirmed by corneal topography and pachymetry.
RESULTS: Fifteen eyes of nine patients (six male and three female) were operated. Mean patient age was 32 years (range 25 to 41 yr). Preoperatively, best spectacle-corrected visual acuity was 20/200 to 20/500 with a range of 12 to 26 D of against-the-rule astigmatism (mean 19.00 D). Follow-up ranged from 13 to 57 months (mean 35 mo). Postoperatively, patients had with-the-rule astigmatism: mean 16.00 D at 6 weeks and 10.50 D at 6 months. In the second postoperative year, mean with-the-rule astigmatism was 4.30 D. Best spectacle-corrected visual acuity was 20/40 in 71% of eyes at final follow-up. No significant complications occurred during the study period.
CONCLUSION: Lamellar crescentic resection was a safe and effective non-penetrating surgical method to manage pellucid marginal corneal degeneration, however, visual recovery was relatively prolonged. [J Refract Surg 2004;20:162-165]
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.