Pars plana vitrectomy, lensectomy, or extraction in transscleral intraocular lens fixation for the management of dislocated lenses in a family with Marfan's syndrome.

摘要

BACKGROUND AND OBJECTIVES: Congenital lens subluxation may be a difficult therapeutic problem. Surgical treatment options include iris manipulation or lens decision, aspiration, intracapsular or extracapsular extraction, and lensectomy through the pars plana. It is not established which kind of aphakic correction is the most appropriate in these cases. PATIENTS AND METHODS: A father and his two sons with Marfan's syndrome were operatively treated for lens dislocation in both eyes. Pars plana vitrectomy was done in all eyes. Dislocated lenses were removed by lensectomy in three eyes and with an intracapsular method in three eyes. The outside-in scleral fixation technique was used for primary posterior chamber intraocular lens (PC IOL) implantation in all cases. RESULTS: All eyes achieved good visual acuity (20/20-20/25). Time of observation ranged between 8 and 20 months. There were no intraoperative or post-operative complications. CONCLUSION: Pars plana vitrectomy and primary scleral-fixated IOL implantation is a safe procedure and gives good visual rehabilitation in adult patients with Marfan's syndrome.