RETINAL-DETACHMENT IN MARFANS-SYNDROME - CHARACTERISTICS AND SURGICALRESULTS

Background: The presence of a rhegmatogenous retinal detachment in a p atient with Mar-fan's syndrome is manifested by narrow pupils, disloca ted lenses, and a spectrum of pathology ranging from simple holes to g iant tears with or without proliferative vitreoretinopathy. Patients a nd Methods: Thirteen patients (18 eyes) with Marfan's syndrome underwe nt surgery for retinal detachment. Characteristic findings were a reti nal detachment in three or more quadrants (12 eyes), a single tear sma ller than 30 degrees (eight eyes), a tear between 80 degrees and 120 d egrees (five eyes), equatorial and postequatorial tears (11 eyes), and advanced proliferative vitreoretinopathy (seven eyes). Nine uncomplic ated retinal detachments were managed with scleral buckling, and nine complicated retinal detachments were managed with pars plana vitrectom y, scleral buckling, and retinal tamponade, mostly with silicon oil Re sults: The results of surgery varied, depending on the nature of the r etinal tear and the presence of proliferative vitreoretinopathy. Compl ete retinal reattachment was achieved in 89% of uncomplicated retinal detachments and in 56% of complicated retinal detachments. Additional partial anatomic success was achieved in two eyes with complicated ret inal detachments where the macula was attached. Visual acuity improved significantly in five eyes with uncomplicated retinal detachments (me dian final vision, 20/80) and in six eyes with complicated retinal det achments (median final vision, 20/200). Conclusion: The results of sur gical treatment for retinal detachments in patients with Marfan's synd rome were comparable with those in patients without Marfan's syndrome. In seven cases of retinal detachment in patients with Marfan's syndro me, we were able to reattach the retina without removing the dislocate d intraocular lens.