PARS PLANA VITRECTOMY FOR THE TREATMENT OF VITREOUS AMYLOID IN PATIENTS WITH HEREDITARY TRANSTHYRETIN AMYLOIDOSIS

PURPOSE: To describe a series of patients with hereditary transthyretin amyloidosis with vitreous amyloid and to study the efficacy and safety of pars plana vitrectomy for its treatment. METHODS: Retrospective study of 266 patients with hereditary transthyretin amyloidosis because of Val30Met mutation submitted to pars plana vitrectomy for vitreous amyloid, with a minimum of 3-month follow-up. Indications for surgery were disabling myodesopsia or two lines loss in visual acuity. Only the first operated eye was considered for analysis. RESULTS: Male patients were operated at younger age (51.0 vs. 53.6, P < 0.001). Best-corrected visual acuity improved from 0.38 to 0.89 (decimal scale, P < 0.001). Preoperative glaucoma was associated with lower gain in visual acuity ( P < 0.001). During the follow-up, 69%, 22%, and 1% developed new-onset glaucoma, retinal angiopathy, or retinal detachment, respectively, and 36% required cataract surgery. Pars plana vitrectomy was also required in the fellow eye in 57%. Hereditary transthyretin amyloidosis-related death occurred in 27%, 9.3 (95% confidence interval 8.0 to 10.7) years after pars plana vitrectomy. CONCLUSION: Vitreous opacities are frequently the first symptomatic manifestation of ocular amyloidosis. Moreover, they may be a marker of mortality. Vitrectomy is a safe and effective treatment, but these patients require long-term follow-up to monitor the development or worsening of glaucoma or retinal angiopathy.