Abstract
Background: Surgical treatment of ectopia lentis in Marfan syndrome cases represents a great challenge for ophthalmic surgeons due to zonular weakness, capsular instability and postoperative aphakia correction. We report three cases of surgical treatment of ectopia lentis due to Marfan syndrome and our approach to aphakia correction by implantation of the retroiridally fixated iris-claw intraocular lens. Methods: Prospective interventional case series. All patients underwent extraction of the subluxated lens with or without combined vitrectomy. Aphakia correction was performed using the Iris-claw intraocular lens, positioned retroiridally by traditional enclavation of both haptics into iris midperiphery. Patients were followed-up for 6 months. Intraocular lens power was calculated using the SRK-T formula. Diagnosis of Marfan syndrome was confirmed in all cases by molecular diagnosis. Genomic DNA from peripheral blood samples of the patients and their relatives was isolated and screened for fibrillin-1 gene mutations by PCR. Results: In all cases Iris-claw intraocular lens implantation was carried out uneventfully. In the postoperative period intraocular lens was stable and correctly centered. There were no signs of excessive or prolonged inflammation or any other complications. Intraocular pressure was normal. Conclusion: Our results suggest that the retroiridally fixated Iris-claw intraocular lens is a very attractive alternative in cases lacking capsular support. It is safe and offers maximal aesthetical and functional results since visual acuity was significantly improved in all patients. However, further evaluation with longer follow-up of a bigger population is desirable.
Highlights
Marfan Syndrome (MFS) is a disorder of the connective tissue
It is safe and offers maximal aesthetical and functional results since visual acuity was significantly improved in all patients
There are more than 1,000 mutations in the human fibrillin-1 gene (FBN-1) that can result in Marfan syndrome
Summary
Marfan Syndrome (MFS) is a disorder of the connective tissue. In 1876, E. There are more than 1,000 mutations in the human fibrillin-1 gene (FBN-1) that can result in Marfan syndrome. FBN1 gene mutations in MFS reduce the amount of fibrillin-1, leading to a severe reduction in the capacity to form microfibrils which in turn alters the structure and integrity of the ECM. In the presence of FBN1 mutations, the reduction in the number of microfibrils in combination with and excessive amount of activated growth factors, lead to a diminished elasticity and tissues instability with the resulting clinical features of MFS, characterized by defects in multiple organs. The most serious symptoms in MFS are associated with the involvement of the cardiovascular system Such symptoms result of an inadequate circulation due to cystic medial degeneration causing prolapse of the mitral or aortic valves; a dilated. We report three cases of surgical treatment of ectopia lentis due to Marfan syndrome and our approach to aphakia correction by implantation of the retroiridally fixated iris-claw intraocular lens
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