engleski

ORBITAL AMYLOIDOSIS – LONG TERM FOLLOW UP

Key words: Purpose: To present echographic characteristics of two stages of development of bilateral orbital involvement in primary systemic amyloidosis in 10-year follow up of a case. Design: Interventional case report Methods: A 50-year-old white female presented to us for the first time in 1985. with the mass and the swelling in the lateral corner of the right eye that enlarged in next five years and, in addition, a masses in the upper medial and lateral corner of the left eye occurred. Biopsy revealed amyloid deposition. Further thorough diagnostic procedure disclosed systemic amyloidosis. The patient presented again to us in January 2000, with large nasal left upper palpebro-bulbar mass that produced marked reduction of ocular motility in all directions and ptosis covering the pupil. The mass was removed and the lid reconstructed. Direct and transbulbar echography, pathohistological analysis, including staining with Congo red and examination under a polarizing microscope for green birenfrigence as well as immunohistochemistry were used to confirm the diagnosis of amyloid. Main Outcome Measures: the size and echo reflectivity of palpebral and orbital lesions, pathohistological and immunohistochemical characteristics of the tissue samples. Results: Echographic examination of the orbits performed in 1990. showed an epibulbar and parabulbar low-reflectivity orbital mass, 12x7 mm, in upper temporal part of the right orbit. A widened right lateral rectus muscle of low reflectivity was also documented. 10 years later direct echography of medial part of the left upper lid discloses palpebral extension of orbital mass. It is 1,7x1,3 mm massive lesion, of irregular, inhomogenous, medium to high reflectivity having rough granular structure. Two small areas, 5 and 3 mm in diameter are seen with high reflectivity that resembles to calcification. Transbulbar echography revealed changes in both orbits. There is widening of the orbital fat echo with the higher reflectivity. The retrobulbar fat close to sclera has reflectivity almost comparable to the scleral. All extraocular muscles are enlarged, including insertions. The muscle sheets are thickned, widened with easily detected higher inner reflectivity than in the mucle itself. There is irregular, inhomogenous, medium to high relectivity of the muscles with scarce calcification. These muscles are more easily seen in the relatively high reflective orbital fat. Conclusion: The initial stage of orbital amyloidosis is characterized with low reflectivity due to acellular aggregates in adipose tissue, walls of small blood vessels and extraocular muscles. The granulomatous foreign body response of the host tissue that probably developed inhomogenously increased the mass reflectivity and caused calcifications.

orbital amyloidosis; echography; palpebro-bulbar mass

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