Retained macular function in X–linked retinoschisis after posterior progression of bullous peripheral schisis

Abstract

Abstract: : Purpose: To demonstrate retained macular function and fixation behavior in boys with retinoschisis who underwent vitrectomy and inner layer retinectomy after progression of bullous peripheral schisis through the fovea. Methods: Retrospective review of interventional case series operated at a pediatric retina referral center over the past three years. Results: Four patients underwent lens–sparing pars plicata vitrectomy for non–clearing vitreous or intra–schisis hemorrhage and were documented to have macula–involving peripheral schisis by pre–operative ultrasound and intra–operative examination (with fluorescein angiography in 2). All had complete removal of the posterior hyaloid and the associated inner layer, with laser retinopexy of the margins of the retinectomy and gas or oil tamponade. After treatment of amblyopia, all patients regained reliable fixation and acuity of 20/200 or better at six months post–operative. Post–operative OCT imaging of the macula in one case suggested retained ganglion cell and nerve fiber layers. Conclusions: In some cases of x–linked schisis, posterior extension of hemorrhagic peripheral schisis occurs predominantly at the level of the internal limiting membrane, and may not be associated with irreversible loss of macular function. While posterior progression of peripheral schisis remains an indication for rapid surgical intervention, some cases will retain macular vision despite migration through the macula. OCT may help to define these cases.

Keywords: retinal degenerations: hereditary • retinal detachment • macula/fovea