Purpose To assess the anatomical and functional results of vitreoretinal surgery in patients with advanced familial exudative vitreoretinopathy (FEVR).
Methods Retrospective analysis of data for 10 eyes of 9 patients with advanced FEVR who underwent pars plana vitrectomy from March 1997 to May 2015 and had a follow-up of at least 12 months. The primary outcomes were final visual acuity (VA) and anatomical success.
Results The average age at the time of the surgery was 10.1±6.5 years (range 2 months–18 years). The mean follow-up period was 58.4±75.1 months. The male-to-female ratio was 7/2. The mean number of vitreoretinal operations was 1.5. At the last visit, 7 (70%) eyes had complete or partial retinal attachment. Preoperatively, the mean Snellen VA was 20/4000 (n=8), and in the remaining 2 (20%) eyes it was recorded as ‘central, steady and maintained’. At final examination, the mean Snellen VA was 20/330 (n=7), and in the remaining 3 (30%) eyes it was recorded as light perception. At the final visit, 5 eyes (50%) had improved VA, 2 eyes (20%) showed stabilisation, and 3 eyes (30%) with total retinal detachment had a decrease in VA. No progression to glaucoma was observed and no enucleation was necessary.
Conclusions Advanced FEVR in young children tends to be more aggressive, leading to severe complications. These cases are challenging and require special consideration. Despite surgery, disease-related complications remain high. However, surgical intervention for advanced FEVR might be of benefit in helping to preserve vision.
- Treatment Surgery
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Contributors MH and MK were involved in the conception and design of the study. MH, MK and SA were involved in the analysis, interpretation and critical revision of the article. MH was involved in the drafting of the manuscript. MH, MK, ISM, MGE and SA were involved in the final approval of the article. MH, ISM and MGE were involved in the data collection and literature research.
Competing interests None declared.
Ethics approval The study protocol was approved by the Ethics Committee of Sisli Memorial Hospital, Istanbul.
Provenance and peer review Not commissioned; externally peer reviewed.