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. DESIGN: A retrospective survey of consecutive patients with X-linked retinoschisis who underwent surgery at our institution during a 16-year period. SETTING: A tertiary-care eye hospital. PATIENTS: Six eyes of four 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 vitrectomy. 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 detachment (one eye), and vitreous hemorrhage (two eyes). The surgical approach was scleral buckling for retinal detachment and vitrectomy for vitreous hemorrhage or proliferative vitreoretinopathy. Anatomic success 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 vitrectomy. Proliferative vitreoretinopathy with retinal detachment was the major reason for reoperation. CONCLUSIONS: Surgery for X-linked retinoschisis-associated retinal detachment and vitreous hemorrhage can yield favorable anatomic and functional results. Multiple operations and the use of advanced vitreoretinal techniques to manage proliferative vitreoretinopathy-related complications, however, were necessary for ultimate success in certain cases.