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Akira Kimura, MD, PhD; Eiko Ando, MD, PhD; Mikiko Fukushima, MD, PhD; Takahisa Koga, MD; Akira Hirata, MD, PhD; Kazue Arimura, MD; Yukio Ando, MD, PhD; Akira Negi, MD, PhD; Hidenobu Tanihara, MD, PhD

Arch Ophthalmol. 2003;121:351-356.

Objective  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 best-corrected 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, 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 in which the onset of glaucoma occurred following amyloid deposition along the pupil, the mean ± SD period between the onsets of pupillary amyloid deposition and glaucoma was 2.55 ± 1.43 years (range, 0.2-4.0 years). Further statistical analyses revealed significant relationships between the onset of secondary glaucoma and both amyloid deposition (P<.001) and vitreous opacity (P<.001). Surgical treatment was required in 15 (75%) of the 20 glaucomatous eyes. In 9 (81%) of the 11 eyes that underwent trabeculectomy, the intraocular pressure was well controlled at or lower than 20 mm Hg during the follow-up period. In the eyes that underwent combined trabeculotomy and sinusotomy (2 eyes), nonpenetrating trabeculectomy (1 eye), or a cyclodestructive procedure (1 eye), the intraocular pressure was poorly controlled.

Conclusions  Glaucoma is not a rare condition in patients with FAP, especially because liver transplantation now enables patients with FAP to live longer. Careful observation of amyloid deposition along the pupil allows the prediction of glaucoma onset.

From the Departments of Ophthalmology (Drs Kimura, Fukushima, Koga, Hirata, Arimura, and Tanihara) and Laboratory Medicine (Dr Y. Ando), Kumamoto University School of Medicine, and the Department of Ophthalmology, Nishi-Nihon Hospital (Dr E. Ando), Kumamoto, Japan; and the Department of Ophthalmology, Kobe University School of Medicine, Kobe, Japan (Dr Negi). Drs Kimura, E. Ando, Fukushima, Koga, Hirata, Arimura, Y. Ando, Negi, and Tanihara have no relevant financial interest in this article.

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