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Abstract #122077 Published in IGR 25-1
Pars-plana vitrectomy for the treatment of vitreous amyloid in patients with hereditary transthyretin amyloidosis
Marques JH; Morais I; Coelho J; Severo M; Menéres MJ; Beirão JMRetina (Philadelphia, Pa.) 2024; 0:
PURPOSE: To describe a series of patients with hereditary transthyretin amyloidosis (ATTRv) with vitreous amyloid and to study the efficacy and safety of pars plana vitrectomy (PPV) for its treatment. METHODS: Retrospective study of 266 patients with ATTRv due to Val30Met mutation submitted to PPV for vitreous amyloid, with a minimum 3-month follow-up. Indications for surgery were disabling myodesopsia or 2 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. PPV was also required in the fellow eye in 57%. ATTRv-related death occurred in 27%, 9.3 95%CI 8.0-10.7 years after PPV. 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.
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