A 19-year-old healthy Indian female noted blurred vision, redness, and pain in the right eye (RE) for 6 months. On examination, visual acuity (VA) was counting fingers in RE and 20/20 in left eye (LE). The RE demonstrated shallow anterior chamber with 360 degrees peripheral anterior synechiae. Approximately 400 translucent spherules of 50-200 micron diameter were embedded in the iris stroma and anterior chamber angle [Fig. and ]. Other findings included posterior subcapsular cataract, elevated intraocular pressure (IOP) of 30 mm Hg, and advanced glaucomatous damage with C:D ratio of 0.9:1. The LE was normal. Iris biopsy demonstrated acellular, amorphous hyaline deposits within the iris stroma, weakly staining with Congo red, suggestive of amyloid []. Transmission electron microscopy (TEM) confirmed amyloid fibrils []. Results of analysis with in situ hybridization for subtyping were inconclusive due to the presence of heavily melanized melanocytes. Systemic evaluation including complete blood count, liver and kidney function tests, serum and urine protein electrophoresis, serum and urine light chain protein, bone marrow biopsy, and PET CT scan were normal. Over the following 3 years, the patient developed cataract, uncontrolled glaucoma, and corneal decompensation, requiring cataract surgery, glaucoma valve surgery, and penetrating keratoplasty, with final VA at 20/30 in affected eye [Fig. and ].
A 70-year-old Caucasian male was diagnosed with iris deposits and elevated IOP LE. On examination, VA was 20/25 RE and 20/30 LE. IOP was 10 mm Hg RE and 16 mm Hg LE, on topical anti-glaucoma medication LE. Gonioscopy revealed ~200 translucent spherules of 200–400 microns diameter in the angle LE [Fig. and ]. Glaucomatous optic disc cupping with superior and inferior arcuate field defects were noted LE. Fine needle aspiration biopsy revealed amorphous material with mild Congo red staining, consistent with amyloid []. Systemic evaluation was normal. Medication compliance was poor with eventual glaucoma progression and refusal of surgical intervention. At 10-year follow-up, phthisis bulbi with no light perception was noted. The RE examination was normal all throughout.