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.