Pars-plana vitrectomy for the treatment of vitreous amyloid in patients with hereditary transthyretin amyloidosis.

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. 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. 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. 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.

Citation

João Heitor Marques, Inês Morais, João Coelho, Milton Severo, Maria João Menéres, João Melo Beirão. Pars-plana vitrectomy for the treatment of vitreous amyloid in patients with hereditary transthyretin amyloidosis. Retina (Philadelphia, Pa.). 2024 Aug 09

PMID: 39121491

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