Amyloidosis of the Orbit and Adnexae

Abstract

Amyloidosis can present to an Occuloplastic clinic in a wide variety of ways. Definitive diagnosis is based on the histopathological findings. Management is often challenging. Multi-disciplinary team involvement is critical in view of its systemic associations.

Lacrimal Gland Amyloidosis: A Clinicopathological Correlation of a Rare Disorder and Review of Literature

“…Electrophoresis may reveal any monoclonal gammopathy and helps with immunoglobulin kappa:lambda ratios. [12][13][14][15] Occasionally multiple myeloma and lymphoproliferative lesions may be associated with lacrimal gland amyloidosis, which warrants systemic skeletal survey and chest and abdomen imaging with or without additional visceral biopsies. 12,13 Most have a primary localized disease that can be excised, debulked, or observed if there is no evidence of a systemic disease, but there appears to be a role of external beam radiotherapy if there is documented progression or recurrence.…”

“…The deposits are congophilic and give the characterisitic apple-green birefringence under intense unidirectional polarized light. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Bony trabeculae with or without marrow elements may be noted corresponding to the intralesional calcification noted radiologically.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Localized amyloidosis is an infrequently encountered disease but has an affinity to mucous membranes like the conjunctiva. 8,10,13 Ocular amyloidosis is most commonly seen involving the conjunctiva and the lid.…”

Insight into the Protein Composition of Immunoglobulin Light Chain Deposits of Eyelid, Orbital and Conjunctival Amyloidosis

“…Amyloid deposits in the conjunctiva and orbit are usually localized and not part of a systemic disease, whereas eyelid involvement may be associated with both local and systemic amyloidosis [24, 25]. The presentation of amyloidosis in the ocular tissues is highly variable making the diagnosis and treatment challenging.…”

Idiopathic Lymphoplasmacellular Mucositis-Dermatitis of the Eyelid

“…8 Localized AL of the orbit can be monitored or treated with surgical debulking and/or radiotherapy. [8][9][10][18][19][20] The initial radiographic features of amyloidosis are nonspecific, often resembling an inflammatory process. Orbital amyloidosis can be unifocal or multifocal and involve one or both orbits on CT. [21][22][23] If the lacrimal gland is involved, calcification may be present.…”