Excision of extensive subretinal fibrosis associated with rhegmatogenous retinal detachments in patients with familial exudative vitreoretinopathy.

Purpose: To determine the characteristics and treatment of the extensive subretinal fibrosis (ESF) in eyes with rhegmatogenous retinal detachment (RRD) but without a tractional retinal detachment in patients with familial exudative vitreoretinopathy (FEVR).

Methods: Ten eyes of 9 men and 1 woman with ESF in patients with RRD-FEVR were studied. The characteristics, surgical procedures, and the outcomes after the removal of the ESF were determined. The ESF was excised by either stripping the ESF from small retinotomies or by en bloc excision through a >180° retinotomy or retinectomy at the ora serrata.

Results: The median age of the patients was 12.5-years. All cases had a unilateral macula-off retinal detachment with a total detachment in 4 eyes and detachment over 2 quadrants in 6 eyes. The first case underwent ESF stripping through small retinotomies that required 7 additional surgeries with a final visual acuity (VA) of hand motion. The subsequent 9 cases underwent en bloc excision resulting in a retinal reattachment with silicone oil tamponade. The silicone oil was removed without a recurrence of the RD during a median follow-up period of 7 years. The median final VA was 20/66 with a range of 20/17 to 20/500.

Conclusion: ESF can be found in FEVR eyes with a slowly progressive RRD. We conclude that the en bloc excision of the ESF is an effective and safe procedure to reattach the retina and thus improve the visual acuity.