Localized orbital amyloidosis - A varied presentation

Abstract

Localized orbital amyloidosis is rare, usually slowly progressive and benign disorder. The most common signs and symptoms include visible periocular mass, ptosis, proptosis, globe displacement, ocular motility disturbances, recurrent periocular subcutaneous hemorrhages, and dry eyes. Herein, we report a case of localized recurrent orbital amyloidosis with strabismus, restricted eye movement, ptosis, and orbital mass as the presentation in a 60-year-old female and managed with debulking and strabismus surgery, resulting in a good cosmetic and functional outcome.

Keywords: Debulking; orbital amyloidosis; recurrence; strabismus surgery.

Figure 1

(a) External photograph showing bumpy swelling involving left upper lid producing severe ptosis (black arrow). (b) Nine gaze photograph showing large left hypotropia with 3 elevation limitation in the left eye

Figure 2

(a) Axial and coronal magnetic resonance imaging showing ill-defined soft tissue seen beneath the left tarsal plate and superior periocular region around insertion of enlarged superior rectus. (b) Intraoperative photograph of the excised mass sent for histopathology analysis. (c-e) HPE stain showing pale pink, amorphous extracellular, glassy, hyaline eosinophilic amyloid-like substance material mostly deposited in and around vessels (black arrows). (f and g) Congo red stain showing red color amyloid deposits (black arrows). (h) Congo red-stained tissue visualized under polarized light showing apple-green birefringence (black arrow)

Figure 3

Postoperative photograph showing improvement in ptosis and residual small left hypotropia