జర్నల్ ఆఫ్ జెనెటిక్ సిండ్రోమ్స్ & జీన్ థెరపీ
అందరికి ప్రవేశం

నైరూప్య

Retropupillary Iris-Claw Intraocular Lens in Ectopia Lentis Due to Marfan Syndrome

Matteo Forlini, Ana Laura Gramajo, Robert Rejdak, Agata Prokopiuk, Oxana Levkina, Adriana IonelaBratu, Paolo Rossini, Perfecto R Cagampang, Gian Maria Cavallini and Cesare Forlini

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.