Abstract

SO.08.09

Amyloidosis – a rare differential diagnosis of an orbital tumor

Sabine Naxer, Abed Atili, Michael Schittkowski
Bereich Strabologie, Neuroophthalmologie und oculoplastische Chirurgie, Abteilung Augenheilkunde, Universitätsmedizin Göttingen, Göttingen

Objective
The differential diagnoses of orbital tumors are numerous; a localised orbital amyloidosis is therefore a rare reason.
Methods
A review of literature about localised orbital amyloidosis will be given. Signs and symptoms are explained by presenting 2 cases.
Results
A 49-year-old woman presents with a slow progressive unilateral ptosis, proptosis and double vision. A 70-year-old man complained of a slowly progressive ptosis over 4 years and about a swelling of the upper eyelid. During the excisional biopsy we found brownish structures that tend to separate into simpler compounds. The histological examination and positive staining with congo red showed in both cases amyloid deposits with considerable signs of inflammation. There was no systemic manifestation of the amyloidosis. After macroscopic tumor excision both patient were recovered. The 49-year-old woman still suffered from ptosis which might be caused by tumor related atrophy of the levator complex. A frontalis suspension procedure was necessary. Both patients are without recurrence over 13 respectively 9 month time.
Conclusions
A specific therapy of this local slowly progressive disease does not exist. The mass of the tumor can be reduced operatively to improve symptoms. The prognosis is good. A systemic manifestation of the amyloidosis has to be excluded every time. Orbital involvement by systemic amyloidosis is described in literature more often and can be the first sign. Therapeutic methods to treat a systemic amyloidosis do exist but the prognosis is pure.

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