To elucidate the clinical features and surgical outcomes of the treatment of secondary glaucoma associated with transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP).Design: Retrospective case study.Participants: Forty-nine Japanese patients with FAP.Methods: For all patients, measurement of bestcorrected visual acuity, intraocular pressure, and visual fields as well as slitlamp and ocular fundus examinations were conducted and compared. In addition, the exact mutation of the amyloidogenic TTR variants was analyzed for all 49 patients with FAP. The TTR mutations included amyloidogenic TTR (ATTR) Val30Met in 41 patients, ATTR Tyr114Cys in 6, ATTR Ser50Ile in 1, and a compound heterozygous mutation of ATTR Val30Met + Arg104His in 1.
Results:The onset of secondary glaucoma was defined as elevation of intraocular pressure and glaucomatous changes in visual field defects. Secondary glaucoma was detected in 12 (24%) of the 49 patients. The incidence of secondary glaucoma in patients with the Val30Met mutation (17%) was lower than for the other FAP genotypes (P=.02 using the 2 test). Of 20 glaucomatous eyes,
The frequency of NVG in PDR eyes within 1 year after vitrectomy was estimated as 7.1%. The risk is independently associated with male sex, younger age, higher baseline IOP, preoperative neovascularization in the angle, and NVG in the fellow eye.
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