Purpose: To review the surgical outcome of autologous plasmin enzyme-assisted vitreoretinal surgery in managing complications associated with congenital X-linked retinoschisis (CXLRS).
Methods: Medical records of 20 patients (22 eyes) with CXLRS complications, treated with autologous plasmin enzyme-assisted vitreoretinal surgery, were reviewed. Surgical techniques were adapted according to a new CXLRS classification.
Results: Median age of the cohort was 3.5 years. Indications for surgical intervention included 8 eyes with schisis involving or threatening the macula, 7 eyes with rhegmatogenous retinal detachment, 4 eyes with tractional retinal detachment, 1 eye with vitreous and intraschisis hemorrhage, 1 eye with obstruction of the macula by an overhanging bullous schisis cavity, and 1 eye with macular pucker. Ninety-one percent (20/22) of eyes had retinal attachment postoperatively after an average of 1.3 procedures per eye. Of the eyes in which visual acuity was measured, 53% (8/15) improved, 33% (5/15) had no change, and 13% (2/15) lost vision.
Conclusion: Plasmin enzyme-assisted vitreoretinal surgery is a safe and effective method for managing the complications associated with CXLRS. Most patients had improved or stable postoperative visions.