Clinical Characteristics and Long-term Visual Prognosis of Familial Exudative Vitreoretinopathy.

Abstract

OBJECTIVE To investigate the clinical characteristics and long-term visual outcomes of familial exudative vitreoretinopathy (FEVR). DESIGN Retrospective case series. SUBJECTS Patients diagnosed with FEVR and treated at Ramathibodi Hospital, Bangkok, Thailand, between January 2007 and February 2024. METHODS Medical records were reviewed for demographic data, clinical features, management, and visual outcomes. Logistic regression was used to identify predictors of visual outcomes. MAIN OUTCOME MEASURES Clinical characteristics, treatment modalities, surgical success, and visual outcomes. RESULTS Ninety-two eyes of 46 patients were included. The median (interquartile range) age of onset was 6.4 (2.0-24.0) months. FEVR stages 1, 2, 3, 4, and 5 were observed in 38.0%, 13.0%, 5.5%, 26.9%, and 16.3% of eyes, respectively. Retinal vessel abnormalities (55.4%) were the most common posterior finding. Overall, 62.0% of eyes required no treatment, 34.8% underwent laser photocoagulation, and 7.6% received surgery (pneumatic retinopexy [PR], pars plana vitrectomy with silicone oil [PPV+SO], or scleral buckling). The surgical success rate was 42.9%. Peripheral avascular retina <9 clock-hours was associated with better visual outcomes (odds ratio [OR], 3.61; 95% confidence interval [CI], 1.06-12.32). Disc dragging, macular dragging, and retinal detachment were associated with worse outcomes, with ORs (95% CI) of 0.12 (0.04-0.36), 0.04 (0.01-0.15), and 0.03 (0.01-0.12), respectively. Stage 4 disease independently predicted poor outcome (adjusted OR, 0.05; 95% CI, 0.00-0.82; p = 0.036). CONCLUSIONS Most patients with FEVR presented with mild disease requiring no treatment, Despite successful treatment, advanced stages (≥4) were significantly associated with poor vision. When appropriately selected, PPV+SO and PR have potential benefits, particularly in achieving anatomical attachment.