Visual and surgical outcomes of retinal detachment after Lens removal for ectopia lentis in pediatric patients with Marfan syndrome.

Abstract

Purpose: To evaluate surgical and visual outcomes following retinal detachment (RD) repair after lensectomy in pediatric patients with Marfan syndrome.

Methods: This retrospective cohort study included patients under 21 years of age with Marfan syndrome who RD following lensectomy for ectopia lentis between January 2011 and January 2024. All patients underwent a comprehensive ophthalmologic assessment at baseline, defined as the visit at which RD was diagnosed, as well as at follow-up visits. Data were collected on clinical features of RD, anatomical success rates, complications, and changes in visual outcomes after surgery.

Results: A total of 33 eyes from 25 patients (18 males, 72%) with a mean age at presentation of 12.6 ± 4.7 years were included. The mean axial length was 27.8 ± 2.8 mm, and the mean postoperative follow-up duration was 6.1 ± 5.8 years. The most common RD mechanisms were horseshoe tears (33%), retinal holes (33%), and giant retinal tears (15%). Fifteen eyes underwent scleral buckle (SB) surgery as the initial procedure (45.5%), 10 underwent pars plana vitrectomy (PPV) alone (30.3%), and 8 underwent combined PPV and SB surgery (24.2%). Complete anatomical success was achieved in 73.0% (24 eyes) of cases: after one surgery in 16 eyes (67%), two surgeries in 6 eyes (25%), and three surgeries in 2 eyes (8%). An additional 12% (4 eyes) remained attached under silicone oil tamponade at final follow-up. Only the presence of proliferative vitreoretinopathy (PVR) was associated with a lower success rate (p = 0.001). Visual acuity significantly improved after surgery (1.7 ± 1.3 LogMAR vs. 0.8 ± 1.1; p < 0.001). Postoperative complications included ocular hypertension (27.3%), corneal edema (18.2%), and cystoid macular edema (9.1%).

Conclusions: Surgical management of RD following lensectomy in pediatric patients with Marfan syndrome remains challenging but is indicated, as it can provide meaningful visual improvement and anatomical stabilization in a subset of cases. The presence of PVR affects both surgical outcomes and the number of surgeries required.