SURGICAL-MANAGEMENT OF COMPLICATIONS ASSOCIATED WITH X-LINKED RETINOSCHISIS

Objective: To evaluate the role of surgical intervention in cases with severe, vision-threatening complications of X-linked retinoschisis. D esign: A retrospective survey of consecutive patients with X-linked re tinoschisis who underwent surgery at our institution during a 16-year period. Setting: A tertiary-care eye hospital. Patients: Six eyes of f our patients were identified. The mean age of the patients at the time of the first surgical procedure was 4.9 years (range, 18 months to 9 years). Intervention: Scleral buckling procedure or pars plana vitrect omy. Main Outcome Measure: Surgical indications and long-term anatomic and visual outcome. Results: Patients were initially operated on for rhegmatogenous retinal detachment (three eyes), exudative retinal deta chment (one eye), and vitreous hemorrhage (two eyes). The surgical app roach was scleral buckling for retinal detachment and vitrectomy for v itreous hemorrhage or proliferative vitreoretinopathy. Anatomic succes s and ambulatory vision (20/400 or better) was achieved in five of the six eyes with a mean follow-up of 3.8 years (range, 1 to 6 1/2 years) . An average of 1.8 procedures per eye were performed. Two of the four eyes approached by primary scleral buckling eventually required vitre ctomy. Proliferative vitreoretinopathy with retinal detachment was the major reason for reoperation. Conclusions: Surgery for X-linked retin oschisis-associated retinal detachment and vitreous hemorrhage can yie ld favorable anatomic and functional results. Multiple operations and the use of advanced vitreoretinal techniques to manage proliferative v itreoretinopathy-related complications, however, were necessary for ul timate success in certain cases.