A 60-year-old man presented with a 2-year history of bilateral, progressive, and painless proptosis and left blepharoptosis. An examination revealed diffuse, firm, nontender masses in both superior orbits. His upgaze was limited in both eyes. There was bilateral resistance to ocular retropulsion and the exophthalmometry was 24 mm. There was a salmon-colored area under the left superior conjunctiva (Figure). The remainder of the ocular examination results were unremarkable. Computed tomography of the orbits showed bilateral, ill-defined soft tissue density lesions present in the intraconal and extraconal spaces. These lesions showed moderate-contrast enhancement and calcific foci. Based on these findings, a working diagnosis of bilateral orbital lymphoma was made and an incisional biopsy was performed. Histopathology results of the specimen showed eosinophilic, hyaline extracellular material that was stained with Congo red and showed green birefringence on a polarization microscopy (Figure). Based on the histopathology results and the subsequent systemic evaluation, a final diagnosis of primary orbital amyloidosis was made.
Singh M, Rao KR, Das A. Primary Orbital Amyloidosis. JAMA Ophthalmol. 2018;136(1):e175727. doi:10.1001/jamaophthalmol.2017.5727
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