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C.D. Witherspoon, T.M. Lara, R.E. Morris; Pars Plana Vitrectomy (PPV), Lensectomy (PPL), Scleral–Sutured Intraocular Lens (SSIOL) and Indirect Ophthalmoscopy Laser Cerclage (IDOLC) Prophylaxis for Subluxed Lens in Marfan Syndrome Patients . Invest. Ophthalmol. Vis. Sci. 2005;46(13):5520.
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Purpose: Marfan syndrome patients are at high risk of retinal detachment after surgical repair of lens subluxation. It is therefore common practice to delay subluxed lens removal until visual acuity is seriously reduced and patients are generally left aphakic. The purpose of this study is to evaluate the effectiveness and safety of pars plana vitrectomy, lensectomy, SSIOL and IDOLC prophylaxis as a method of management for lens subluxation in patients with Marfan syndrome. Methods: A restrospective review of medical records of patients with Marfan syndrome and subluxed lenses, who underwent PPV, PPL, SSIOL and IDOLC prophylaxis. Results: A total of 28 eyes (18 patients) were identified. Surgical indications were visual acuity (VA) loss and significant lens subluxation. Mean age was 25.8 yrs. Mean preoperative VA was 20/54 (logmar: 0.37). Mean postoperative VA was 20/28 (logmar: 0.12) at a mean follow up of 26.5 months. A total of 26 eyes (92.7%) had a postoperative VA of 20/60 or better. Of the four eyes in which VA ranged from 20/50 to 20/60, amblyopia was present in 3 of these eyes. Two eyes were from an infant and were unable to obtain a reliable vision. One patient received inadequate IDOLC prophylaxis due to corneal epithelial edema and this may have been responsible for the only retinal detachment (RD) that occurred in this series. This patient developed a subclinical RD early postoperatively that was successfully repaired. Conclusions:Current techniques for PPV, PPL and SSIOL when combined with IDOLC prophylaxis appear to be an effective technique for treatment of crystalline lens subluxation in Marfan syndrome patients, with relatively low risk of retinal detachment.
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