Purchase this article with an account.
A. Kimura, E. Ando, M. Fukushima, T. Koga, A. Hirata, K. Arimura, Y. Ando, A. Negi, H. Tanihara; Secondary Glaucoma in Patients with Familial Amyloidotic Polyneuropathy . Invest. Ophthalmol. Vis. Sci. 2003;44(13):2213.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
Purpose: To elucidate the clinical features and surgical outcomes of treatment of secondary glaucoma associated with transthyretin (TTR) related familial amyloidotic polyneuropathy (FAP). Method: For all patients, measurement of corrected visual acuity, intraocular pressure, and visual field, and slit-lamp and ocular fundus examinations were conducted and compared. In addition, the exact mutation in the amyloidogenic TTR variants was analyzed for all 49 FAP patients. The TTR mutations included amyloidogenic TTR (ATTR) Val30Met in 41 patients, ATTR Thy114Cys in 6, ATTR Ser50Ile in 1, and a compound heterozygous mutation of ATTR Val30Met+Arg104His in 1. Result: 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 Val30Met (17%) was lower than for the other FAP genotypes (p = 0.022, chi-squared test). Of 20 glaucomatous eyes, amyloid deposition on the pupil and anterior surface of the lens was found in 18 eyes. Amyloid deposition was found prior to glaucoma in 11 eyes, and at the first visit to our clinic in another 7 eyes. In the 11 eyes with the onset of glaucoma after amyloid deposition along the pupil, the mean period (+ standard deviation) between the onsets of pupillary amyloid deposition and glaucoma was 2.55 + 1.43 years. Further statistical analyses revealed significant relationships between the onset of secondary glaucoma and amyloid deposition, and vitreous opacity. Surgical treatment was required in 15 (75%) of the 20 glaucomatous eyes. In 9 (81%) of the 11 eyes that underwent trabeculectomy, intraocular pressure was well controlled at or below 20 mmHg during the follow-up period. In the eyes after combined trabeculotomy and sinusotomy (2 eyes), nonpenetrating trabeculectomy (1 eye), or cyclodestructive procedure (1 eye), intraocular pressure was poorly controlled. Conclusions: The occurrence of glaucoma is not a rare condition in patients with FAP, especially since liver transplantation now enables patients with FAP to live longer. Careful observation of the amyloid deposition along the pupil allows prediction of the onset of glaucoma.
This PDF is available to Subscribers Only