Recurrent Retinal Detachment in Stickler Syndrome

TL;DR

This study examines why retinal detachment recurs in Stickler Syndrome patients and finds that early recurrence is often due to PVR, while late recurrence is linked to new retinal breaks.

Contribution

The study identifies distinct clinical patterns and risk factors for early and late retinal detachment recurrence in Stickler Syndrome patients.

Findings

Early recurrent retinal detachment was mostly caused by proliferative vitreoretinopathy (PVR).

Late recurrent retinal detachment was primarily due to new retinal breaks.

Eyes treated with primary scleral buckling remained at high risk for late re-detachment.

Abstract

To assess clinical factors leading to recurrent retinal detachment (RD) and characteristics of recurrence in patients with Stickler Syndrome.

Retrospective case series study of patients with clinical diagnosis of Stickler Syndrome who underwent rhegmatogenous RD repair. Recurrent RD after initial surgery was categorized as “early” if the recurrence was within 1 year or “late” if greater than 1 year.

Thirty eyes from 22 patients underwent rhegmatogenous RD repair. For initial repair, 13 eyes underwent pars plana vitrectomy combined with scleral buckling (PPV/SB), 16 eyes underwent primary scleral buckling (SB), and 1 eye underwent pneumatic retinopexy (PnR). Recurrent RD occurred in 6 (46%) PPV/SB eyes (5 early and 1 late), 10 (63%) SB eyes (3 early and 7 late), and 0 (0%) PnR eyes (p = 0.61). PPV/SB was preferred for eyes presenting with total detachment (82%), giant retinal tears (100%), and proliferative vitreoretinopathy (PVR) (80%). For eyes with early recurrent RD, 6 (75%) developed PVR leading to recurrence. For eyes with late recurrent RD, 7 (87.5%) developed a new retinal break leading to recurrence, including 4 with a break posterior to the buckle indentation apex. At last follow-up, median LogMAR visual acuity was 0.68 for eyes with recurrent RD compared to 0.29 for eyes without recurrence (p = 0.27).

Early recurrent RD was mostly caused by PVR, while late recurrent RD was mostly due to new retinal breaks. Eyes with seemingly uncomplicated rhegmatogenous RD repair with primary SB remained at high risk for late re-detachment.

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