Ocular amyloidosis and secondary glaucoma

Objective: To report the clinical and histopathologic findings in two Gases of secondary glaucoma associated with amyloidosis. Design: Methods: Two case reports. Methods: Retrospective review of clinical findings, course, and treatment o f the two patients. The histopathologic findings from available biopsy mate rial were also reviewed. Main Outcome Measures: Intraocular pressure (IOP), visual field changes, an d surgical outcome. Results: The first case describes a 76-year-old woman with orbital amyloido sis who developed gradual unilateral elevation of IOP that was poorly respo nsive to medical therapy and underwent filtration surgery. Episclera[ venou s pressure was elevated on the affected side, and histopathologic analysis of the conjunctival tissue confirmed perivascular amyloid deposits, further suggesting raised episcleral venous pressure to be a possible mechanism of glaucoma. The second case describes a 47-year-old white woman with familia l amyloid neuropathy with a transthyretin cys-114 mutation, The association of glaucoma with this mutation has not been described previously. Persisti ng elevation of IOP in one eye was initially responsive to topical antiglau coma medications but eventually required filtration surgery. Amyloid partic les were found in the aqueous and on the lens surface. Histopathologic anal ysis of the aqueous and sclerectomy specimens demonstrated amyloid, suggest ing outflow obstruction as a possible mechanism of glaucoma, Conjunctival b uttonholing complicated filtration surgery in both cases, and the leaks eve ntually resolved with good control of IOP. Conclusions: Amyloid associated with glaucoma may involve different pathoph ysiologic mechanisms. The elevated IOP may not respond well to medical ther apy. Cautious surgical manipulation of the conjunctiva is warranted in thes e cases.