Outcomes of vitreoretinal surgery in patients with X-linked retinoschisis.

Background And Objective: To assess the outcomes of vitreoretinal surgery in the treatment of vision-threatening posterior segment complications of X-linked retinoschisis.

Patients And Methods: The authors performed a retrospective analysis of 16 eyes from 11 patients who underwent vitreoretinal surgery. All the patients had a documented positive family history of X-linked retinoschisis, and all patients had bilateral macular disease.

Results: The ages of the patients ranged from 14 months to 37 years (mean age 15.1 years; median age 11.5 years), and postoperative follow-up ranged from 3 months to 10 years (mean 2.8 years; median 1 year). The indications for surgical intervention included rhegmatogenous retinal detachment (12 eyes), vitreous hemorrhage (2 eyes), progression of the schisis cavity through the fovea (2 eyes), cataract associated with a persistent hyperplastic primary vitreous-like condition (2 eyes), and exudative maculopathy (1 eye). The primary surgical intervention included pars plana vitrectomy alone (7 eyes), pars plana vitrectomy and pars plana lensectomy (4 eyes), and a scleral buckle procedure alone (5 eyes). Surgical success (defined as reattachment of the retina, removal of media opacities, or arrest of schisis progression) was achieved in 14 of 16 eyes, after an average of 1.2 procedures per eye. The major reason for reoperations was recurrent retinal detachment due to proliferative vitreoretinopathy. Two eyes were eventually enucleated due to pain associated with neovascular glaucoma resulting from recurrent retinal detachment. Of the remaining 14 eyes, visual acuity improved in 8 eyes and remained unchanged in 6 eyes.

Conclusion: Vitreoretinal surgery is often helpful in stabilizing or improving visual function in patients with posterior segment complications from X-linked retinoschisis.