Bilateral Asymmetric Rhegmatogenous Retinal Detachment in a Patient with Stickler Syndrome

Abstract

Here we present the long-term anatomical and visual outcomes of bilateral asymmetric rhegmatogenous retinal detachment repair in a patient with Stickler syndrome. A 17-year-old girl presented with decreased visual acuity in both eyes for more than one year. Her best-corrected visual acuity (BCVA) was 0.1 in the right eye and 0.05 in the left eye. Slit-lamp anterior segment examination revealed subcapsular cataract in both eyes. Fundus examination showed bilateral rhegmatogenous retinal detachment, chronic retinal detachment accompanied by multiple retinal holes, tears and membranous fibrillary vitreous in the peripheral retina. Grade C2 proliferative vitreoretinopathy was observed in the left eye. Scleral buckling surgery was performed initially for both eyes. After the primary surgical procedure, retinal reattachment was achieved in the right eye and the left eye underwent phacoemulsification, intraocular lens implantation, pars plana vitrectomy (PPV), and silicone oil injection. After these surgical procedures retinal reattachment was achieved in the left eye. Silicone oil removal was performed six months after PPV surgery. After surgical treatment, BCVA was 0.6 in the right eye at the end of the 3.5-year follow-up period. After silicone oil removal, BCVA reached 0.2 in the left eye after 36 months of follow-up and retinal reattachment was achieved in both eyes. Scleral buckling surgery and PPV are effective and confidential methods for the treatment of chronic retinal detachment cases in Stickler syndrome.

Surgical treatment of vitreoretinal complications in a patient with Stickler syndrome type 1: a case report

“…It was first reported in 1965 by Stickler et al [3]. He described ocular findings, auricular, orofacial (isolated or as part of the sequence of Robin), joints changes by defects in collagen type II, IX, and XI [2,[4][5][6]. Its annual incidence is estimated at 1:7.500 to 1:10.000 [5][6][7][8], and it is considered the most frequent cause of non-traumatic retinal detachment in the pediatric population [8].…”