Abstract
Purpose :
Early and recurrent rhegmatogenous retinal detachments (RRD) are a cardinal feature of Stickler Syndrome. We performed a retrospective case series study to assess if the initial surgical procedure had predictable value for the development of recurrent RRD with particular attention to late recurrence.
Methods :
We identified patients with a clinical diagnosis of Stickler Syndrome who had undergone RRD surgery at UPMC from 2006-2021. A recurrent RRD after initial surgery was categorized as “early” if the detachment recurred within 1 year or “late” if greater than 1 year. The study was approved by the University of Pittsburgh’s IRB.
Results :
We identified 30 eyes from 22 patients that underwent RRD repair. The median age at initial RRD was 10 (range 4-40); 16 (72%) were male; 14 (64%) had molecular confirmation. For initial repair, 16 eyes underwent scleral buckling (SB) only, 13 eyes underwent combined pars plana vitrectomy and SB (PPV/SB), and 1 eye underwent pneumatic retinopexy. In SB only, recurrent RRD occurred in 10/16 (63%), 3/10 early and 7/10 late, and in combined PPV/SB, recurrent RRD developed in 6/13 (46%), 5/6 early and 1/6 late (p = 0.47, Fisher’s exact test). The pneumatic retinopexy eye remained attached.
For initial presentation, PPV/SB was preferred for total RRD (9/11, 82%), giant retinal tears (8/8, 100%), and proliferative vitreoretinopathy (PVR) (4/5, 80%). Of those with PVR initially, 3/5 (60%) had early recurrent RRD (2 PPV/SB, 1 SB). However, all recurrent RRD in the PPV/SB group had PVR at recurrence. In the SB only group with recurrent RRD, 4/10 (40%) had a break posterior to the scleral buckle that led to detachment, with all occurring late. For treatment of recurrent RRD in SB only, laser retinopexy was used initially in 3/10, but all eventually required PPV.
Conclusions :
Our study found that there was no significant difference between SB and PPV/SB for initial surgery. However, we did find that SB only had a higher rate of late recurrent RRD with a high number due to breaks posterior to the SB. SB only is the preferred surgical approach for RRD in young patients due to the attached vitreous and clear lens; however, this may not be optimal in Stickler Syndrome due to early vitreous degeneration and an anomalous vitreoretinal interface. Our results may suggest that early PPV in select cases, use of wide band SB indentation, and close follow-up should be further explored to reduce the risk of late recurrent RRD.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.