Dramatic regression of macular and peripheral retinoschisis with dorzolamide 2 % in X-linked retinoschisis: a case report

BACKGROUND: X-linked retinoschisis is one of the more frequently encountered inherited macular retinal disorders affecting young males, causing loss of vision. Patients exhibit macular schisis and peripheral schisis, which can mimic retinal detachment, a very different entity that requires surgical intervention. CASE PRESENTATION: An 8-month-old African-American boy was presented to our hospital with severe X-linked retinoschisis involving symmetrical bullous peripheral retinoschisis in both eyes, mimicking retinal detachment. One eye received multiple surgeries for retinal detachment repair that were complicated by proliferative vitreoretinopathy. Later, portable optical coherence tomography was used to confirm absence of retinal detachment despite a corrugated fundus appearance in the fellow eye. Following treatment with topical dorzolamide 2 % for 18 months, there was dramatic regression of both macular and peripheral schisis cavities in the nonoperative eye. CONCLUSIONS: Severe bullous peripheral schisis in infants with severe X-linked retinoschisis may produce posterior corrugations that mimic rhegmatogenous retinal detachment. Clinical suspicion for retinal detachment in infants with X-linked retinoschisis should be confirmed by portable optical coherence tomography before surgical intervention. Bullous peripheral schisis can remain clinically stable over time, but topical dorzolamide 2 % may facilitate collapse.