Retinal detachment in Marfan syndrome: clinical characteristics and surgical outcome

To describe the clinical characteristics of retinal detachments in patients with Marfan syndrome and report the surgical outcome of vitreoretinal surgery.

Records relating to 53 eyes of 45 patients with Marfan syndrome who underwent surgery for rhegmatogenous retinal detachment were reviewed. Of the 53 eyes, 24 (45.3%) underwent scleral buckling as the first procedure and 29 (54.7%) underwent vitrectomy surgery with scleral buckle as the first procedure. Main outcome measures included clinical characteristics of retinal detachment, reattachment rates, and functional improvement in vision.

Characteristic findings included total retinal detachment in 40 (75.5%) eyes, atrophic holes in 24 (45.3%) eyes, more than four retinal breaks in 21 eyes (39.6%), preequatorial and postequatorial breaks in 20 (37.7%) eyes, giant retinal tears in six (11.3%) eyes, and proliferative vitreoretinopathy (posterior, anterior, or both) in nine (17%) eyes. In 30 (56.6%) eyes, retinal breaks were located only in the temporal half of the retina. Of the 24 eyes with myopia, 13 (54.2%) had a myopic correction greater than 7 diopters. At the median follow-up of 10.7 months, complete retinal reattachment was obtained in 87.6% and 86.2% of patients undergoing scleral buckling (including additional procedures such as vitrectomy) and vitrectomy surgery, respectively. In eyes with reattached retinas, a final visual acuity of 20/200 or better was obtained in 81% of the patients after scleral buckling and in 56% of the patients after vitrectomy surgery (P = 0.07).

Retinal detachment in Marfan syndrome is complete in 75% of the eyes. More than half (56%) the eyes had a retinal break only in the temporal half of the retina, and 83% had at least a break in the temporal half of the retina. Currently available vitreoretinal surgical techniques result in successful reattachment of the retina in approximately 86% of the eyes.