Retinal Detachments in Pediatric Patients with X-linked Retinoschisis: Characteristics and Surgical Outcomes

Abstract

Purpose : Retinal detachments (RDs) are a feared complication of X-linked retinoschisis (XLRS) and portend a poor prognosis. Surgical strategies for repair vary widely, and there are currently no consensus guidelines for management.

Methods : Retrospective analysis of pediatric patients undergoing vitreoretinal surgery between 2000 and 2022 for XLRS-associated RD at the Bascom Palmer Eye Institute. Inclusion criteria were an age less than 18 years, clinical or genetic diagnosis of X-linked retinoschisis, and presence of a rhegmatogenous retinal detachment.

Results : Ten patients (12 eyes) met inclusion criteria. All patients were male, and the average age at presentation was 8 years (range 2-17). The majority of detachments arose inferiorly (67%), two detachments were located superiorly (17%), and two were near total detachments (17%). Seven (58%) detachments were macula-involving. Seven (58%) detachments were repaired with a combined scleral buckling procedure and vitrectomy, three (25%) detachments were repaired with vitrectomy alone, and 1 (8%) underwent a primary scleral buckling procedure. One asymptomatic RD in a fellow eye was observed without surgery due to chronic appearance and lack of progression. Silicone oil tamponade was used in 9 of 10 (90%) cases undergoing vitrectomy, and C₃F₈ gas in 1 (10%) case. Final visual acuity was 20/200 or better in 8 (67%) eyes. Of eyes that underwent surgical intervention, 10 of 11 (91%) remained attached at final follow-up.

Conclusions : Surgical repair in patients with XLRS-associated RDs is associated with a relatively high rate of successful reattachment. Despite favorable anatomic outcomes, visual prognosis after repair may be guarded.

This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.