Retinal detachment in Marfan's syndrome. Characteristics and surgical results

The presence of a rhegmatogenous retinal detachment in a patient with Marfan's syndrome is manifested by narrow pupils, dislocated lenses, and a spectrum of pathology ranging from simple holes to giant tears with or without proliferative vitreoretinopathy.

Thirteen patients (18 eyes) with Marfan's syndrome underwent surgery for retinal detachment. Characteristic findings were a retinal detachment in three or more quadrants (12 eyes), a single tear smaller than 30 degrees (eight eyes), a tear between 80 degrees and 120 degrees (five eyes), equatorial and postequatorial tears (11 eyes), and advanced proliferative vitreoretinopathy (seven eyes). Nine uncomplicated retinal detachments were managed with scleral buckling, and nine complicated retinal detachments were managed with pars plana vitrectomy, scleral buckling, and retinal tamponade, mostly with silicon oil.

The results of surgery varied, depending on the nature of the retinal tear and the presence of proliferative vitreoretinopathy. Complete 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 retinal detachments where the macula was attached. Visual acuity improved significantly in five eyes with uncomplicated retinal detachments (median final vision, 20/80) and in six eyes with complicated retinal detachments (median final vision, 20/200).

The results of surgical treatment for retinal detachments in patients with Marfan's syndrome were comparable with those in patients without Marfan's syndrome. In seven cases of retinal detachment in patients with Marfan's syndrome, we were able to reattach the retina without removing the dislocated intraocular lens.