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Ocular amyloid: adnexal and systemic involvement

ABSTRACT Purpose: To investigate the natural history of ocular adnexal and orbital amyloidosis. Methods: In a retrospective, non-comparative case series, the clinical records of patients with biopsy-proven ocular, adnexal, and orbital amyloidosis managed at our institution between 1980 and 2016 were evaluated. Results: Forty-one patients (29 female; 71%) were identified. The mean interval from presentation to diagnosis was 24 months (median 12 months, range 1–84 months). Whilst most patients presented with a conjunctival mass (34/41; 83%) or ptosis (15/41; 37%), the diagnosis was not immediately evident in all – two patients had 3 ptosis operations prior to obtaining a tissue biopsy that revealed amyloid deposition. Three-quarters (31/41; 76%) of patients had localised primary ocular adnexal and orbital amyloidosis, 4 (10%) had associated systemic disease, and 6 (15%) were found to have underlying haematological malignancy on further investigation. During a mean follow-up of 8 years (median 7 years; range 6 months – 36 years), 2 (5%) patients lost vision, 21 (51%) had surgical intervention other than biopsy, and 2 (5%) had local radiotherapy for amyloid deposition secondary to lymphoproliferative disease. Conclusions: The varied presentations of ocular adnexal and orbital amyloidosis and the need for confirmatory biopsy often leads to a significant delay between first symptoms and diagnosis. While rarely sight-threatening, ocular adnexal and orbital amyloidosis carries significant morbidities and has a systemic association in a quarter of patients.