Outcomes of Pars Plana Vitrectomy in Inherited Retinal Degenerations

To describe the management and outcomes of pars plana vitrectomy (PPV) for common surgical retinal conditions, including epiretinal membrane (ERM), full-thickness macular hole (FTMH), and retinal detachment, in patients with inherited retinal degenerations (IRDs).

Retrospective case series study.

Patients with IRDs who underwent PPV between 2007 and 2024, excluding subretinal injections.

We reviewed patient demographics, clinical information, genetic testing, diagnostic testing, and surgical information.

Surgical outcomes and best-corrected visual acuity (BCVA).

Twenty-six eyes from 25 patients were included. Seven patients (28%) had ERM, 10 patients (36%; 11 eyes) had FTMH, and 9 patients (36%) had other surgical indications. These included progressive bullous retinoschisis, nonclearing vitreous hemorrhage, rhegmatogenous retinal detachment, tractional retinal detachment secondary to Coats-like vasculopathy, persistent macular detachment after subretinal gene or stem cell therapy, and dislocated intraocular lens. The mean follow-up duration was 51 months (range, 3 months to 17 years). Mean age at diagnosis of the surgical condition was 48.2 ± 21.2 years (range, 4-77 years), and 16 patients (64%) were male. At diagnosis, BCVA was worse in eyes requiring surgery (mean 1.19 ± 0.87 logarithm of minimum angle of resolution [logMAR]) compared with fellow eyes (mean 0.65 ± 0.71 logMAR; P = 0.006). At postoperative month 3, BCVA (mean 1.16 ± 1.01 logMAR; P = 0.86) was similar to preoperative values. The BCVA at final follow-up (mean 1.12 ± 1.06 logMAR; P = 0.65) also did not differ significantly from baseline. In ERM eyes, mean central macular thickness improved significantly from 425 ± 158 µm preoperatively to 270 ± 79 µm at postoperative month 3 (P = 0.01). Eyes with better baseline BCVA and more intact ellipsoid zones appeared to achieve better visual outcomes. Among FTMH eyes, topical therapy did not result in hole closure. Surgical closure was achieved in 10 eyes (91%) with 1 surgery, while 1 eye (9%) required 2 procedures.

These data indicate that PPV for common retinal conditions can achieve a high single-surgery anatomic success rate. However, the visual gains achieved in eyes with an IRD are generally smaller than in otherwise healthy eyes, suggesting a conservative approach to patient selection.

The authors have no proprietary or commercial interest in any materials discussed in this article.