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A case of primary localized orbital amyloidosis

Poster Details

First Author: C.Lin TAIWAN

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Abstract Details

Purpose:

Amyloidosis is the abnormal formation of proteins, amyloid fibrins. It is characterized by extracellular deposition in various organs. Isolated orbital amyloidosis is a rare condition and requires systemic examination. We report a case of amyloid deposit in the orbit whose systemic investigation has been negative.

Setting:

Taipei City Hospital, Renai branch, Taipei, Taiwan

Methods:

A 39-year-old woman presented to the eye clinic with sudden onset of left-sided palpable orbital mass. No present of exophthalmos, ptosis or ocular motility limitation. Physical examination revealed normal visual acuity and intraocular pressure. No systemic involvement was noted by systemic workup and it confirmed the primary orbital amyloidosis.

Results:

The patient was subject to full clinical examination, laboratory examinations, orbital echography, magnetic resonance imaging, total body computed tomography scans, and rectal mucosa survey. It was necessary to exclude systemic amyloidosis. Orbital computed tomography demonstrated localized anterior orbital soft tissue infiltration. The presence of amyloid deposits was confirmed by biopsy of orbital mass. The pathology showed marked amphophilic material deposition and focally lymphocytic cell aggregation. Congo red staining revealed light-green birefringence under polarized light, compatible with amyloidosis.

Conclusions:

Orbital amyloidosis is a very rare disease, but it still should be considered in the diagnosis of orbital mass. The treatment usually consists of complete surgical excision of the amyloid mass and follow-up for a likely local recurrence. Systemic workup would be necessary to exclude systemic involvement.

Financial Disclosure:

NONE

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