Resolution of Mid-Peripheral Schisis in X-Linked Retinoschisis with the Use of Dorzolamide

Abstract

X-linked retinoschisis (XLRS) is an early-onset hereditary retinal dystrophy. This disease is caused by mutation(s) of the RS1 gene, which encodes for a protein (retinoschisin) that binds to the external membrane of photoreceptor and bipolar cells and plays a role in the structural integrity of the retinal layers. 1 In the first few decades of life cysticappearing (schitic) lesions are found in the macula, and central visual acuity is decreased. Clinically detectable peripheral retinoschisis, most commonly observed bilaterally in the inferior-temporal retina, is often found in XLRS. Subclinical extramacular cystoid spaces were observed on spectral-domain optical coherence tomography (SD-OCT) in 5 of 11 eyes of XLRS patients in one study. 2 Carbonic anhydrase inhibitors have been found to reduce the macular intraretinal cystoid spaces in a notable number of XLRS patients. 3 In this current report, we present a patient diagnosed with XLRS with intraretinal cystoid spaces in the macula and extending to the midperipheral retina. With the use of topical dorzolamide 2%, the intraretinal cystoid spaces resolved from the macula out to the mid-peripheral retina. To our knowledge, the resolution of mid-peripheral retinal cystoid spaces with the use of a carbonic anhydrase inhibitor has not been previously reported.