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

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.