The purpose of the study was to describe the main clinical and epidemiologic characteristics, treatment options, and outcome in a large series of patients with periocular and orbital amyloidosis. This is a retrospective, descriptive, observational study of a case series of 14 patients with periocular and orbital amyloidosis and is a review of previously published cases with this diagnosis between September 2004 and January 2015.

 

In this study, we analyzed our 14 patients in conjunction with 69 well-documented cases of orbital and/or periocular amyloidosis previously reported, with a total of 83. Of these, 54 were female (65.1 %), 28 male (33.7 %), and one with unspecified gender. The mean age at diagnosis was 54.9 years (range, 18-87). The localization of the amyloidosis was classified as superficial, deep and combined, with involvement of 53 (63.9 %), 26 (31.3 %), and four cases (4.8 %) in each group, respectively. The main findings in superficial amyloidosis were mass or tissue infiltration (84.9 %) and ptosis (30.2 %) and, in the cases with deep involvement, mass (65.4 %), proptosis (57.7 %), limited ocular movements (34.6 %), ocular displacement (30.8 %), and ptosis (26.9 %). The cases with combined involvement presented with signs and symptoms of the two groups.

 

Regarding the outcome, 43 patients were reported stable after the diagnosis and 21 had recurrence or required new surgical procedures. Periocular and orbital amyloidosis is a rare disease that can present with a variety of symptoms and signs depending on the localization and extension of involvement. Its prompt recognition is important in order to investigate systemic disease, which will affect the prognosis of each case.