RE-INTERVENTION IN DE NOVO VITREOUS OPACITIES AFTER PARS PLANA VITRECTOMY IN FAMILIAL AMYLOIDOTIC POLYNEUROPATHY TTR VAL30METPORTUGUESE PATIENTS

Purpose: To report management of de novo vitreous amyloid opacities after previous pars plana vitrectomy in familial amyloidotic polyneuropathy transthyretin Val30Met. Methods: This work is a retrospective observational consecutive case series of five eyes of four patients. Demographic data, transthyretin mutation involved, age at the beginning of disease, duration of disease, treatment (liver transplant or tafamidis), time between vitrectomy and re-intervention, and ophthalmologic changes were evaluated. Surgical re-intervention included phacoemulsification with intraocular lens implantation in phakic eyes, re-vitrectomy as complete as possible with posterior capsulectomy, and internal limiting membrane peeling if wrinkling of internal retinal surface was present. Results: All patients had transthyretin Val30Met mutation, and three were women. Mean age of onset of the disease was 52 ± 11.0 years, and average evolution time of the disease was 8 years. Three patients had been submitted to liver transplant 4, 9, and 15 years before. Time between first vitrectomy and surgical re-intervention was longer than 2 years in all cases. Two eyes had amyloid deposits on anterior lens surface and pupillary border with scalloped pupil. Two eyes were phakic. Glaucoma was present in two eyes; one of them had previous trabeculectomy. All cases had vitreous opacities behind posterior lens capsule and at vitreous base area. After re-intervention, no further recurrence was observed (average follow-up of 10 months). Conclusion: De novo vitreous amyloid opacities may occur several years after pars plana vitrectomy. Amyloid deposition in vitreous cavity was observed only in strong vitreous adherence locations (behind posterior lens capsule and at vitreous base area). The authors expect that this procedure, an extensive re-vitrectomy associated with posterior capsulectomy, will prevent de novo vitreous amyloid opacities.