ORBITAL AMYLOIDOSIS
Keywords:
orbital amyloidosis, echography, palpebro-epibulbar amyloidAbstract
Background. Authors want 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.
Methods. A 65-year old white female with 10-year-long history of orbital involvement in primary systemic amyloidosis presented to us with large nasal left upper palpebro-bulbar mass that produced lateral displacement of the globe, marked reduction of ocular motility in all directions and ptosis covering the pupil. Direct and transbulbar echography, pathohistological analysis, and immunohistochemistry were used to confirm the diagnosis of amyloid. The mass was partially removed and the lid reconstructed.
Results. The echographic examination of the orbits performed in 1990 showed an epibulbar and parabulbar low-reflectivity orbital mass in the upper temporal part of the right orbit. A widened right lateral rectus muscle of low reflectivity was also documented. Ten years later direct echography of the medial part of the left upper lid discloses palpebral extension of the orbital mass. It has irregular, inhomogeneous, medium to high reflectivity with rough granular structure and calcifications. Transbulbar echography revealed changes in both orbits. There is widening of the orbital fat echo with the higher reflectivity. All extraocular muscles are enlarged, including insertions. The widest is the right lateral rectus muscle. The muscle sheaths are thickened, widened with easily detected higher inner reflectivity than in the muscle itself. There is irregular, inhomogeneous, medium to high reflectivity of the muscles with scarce calcification.
Conclusions. The initial stage of orbital amyloidosis is characterized with low reflectivity. Ten years later, the mass reflectivity inhomogeneously increased and calcifications developed.
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References
Murdoch IE, Sullivan TJ, Moseley I, Hawkins PN, Pepys MB, Tan SY, Garner A, Wright JE. Primary localised amyloidosis of the orbit. Br J Ophthalmol 1996; 80: 1083–6.
Friedman NJ, Pineda R, Kaiser PK eds. The Massachusetts eye and ear infirmary illustrated manual of ophthalmology. Philadelphia: WB Saunders Company, 1998: 86–7.
Henderson JW. Orbital tumors. 3rd ed. New York: Raven Press, 1994: 298– 301.
Shields JA, Shields CL eds. Eyelid & conjunctival tumours. Philadelphia: Lippincott Williams & Wilkins, 1999: 174–4.
Ando E, Ando Y, Okamura R, Uchino M, Ando M, Negi A. Ocular manifestations of familial amyloidotic polyneuropathy type I: long term follow up. Br J Ophthalmol 1997; 81: 295–8.
Nelson GA, Edward DP, Wilensky JT. Ocular amyloidosis and secondary glaucoma. Ophthalmology 1999; 106 (7): 1363–6.
Molia LM, Lanier JD, Font RL. Posterior polymorphous dystrophy associated with posterior amyloid degeneration of the cornea. Am J Ophthalmol 1999; 127 (1): 86–8.
Okamoto K, Ito J, Emura I, Kawasaki T, Furusawa T, Sakai K, Tokiguchi S. Focal orbital amyloidosis presenting as rectus muscle enlargement: CT and MR findings. Am J Neuroradiol 1998; 19 (9): 1799–801.
Hill VE, Brownstein S, Jordan DR. Ptosis secondary to amyloidosis of the tarsal conjunctiva and tarsus. Am J Ophthalmol 1997; 123 (6): 852–4.
Massry GG, Harrison W, Hornblass A. Clinical and computed tomographic characteristics of amyloid tumor of the lacrimal gland. Ophthalmology 1996; 103 (8): 1233–6.
Pasternak S, White VA, Gascoyne RD, Perry SR, Johnson RLC, Rootman J. Monoclonal origin of localised orbital amyloidosis detected by molecular analysis. Br J Ophthalmol 1996; 80: 1013–7.
Hubbard AD, Brown A, Bonshek RE, Leatherbarow B. Surgical management of primary localised conjunctival amyloidosis causing ptosis (letter). Br J Ophthalmol 1995; 79 (7): 707–7.
Henč-Petrinović Lj, Dominis M, Petrinović J, Pavičić A. A rare case of bilateral orbital amyloidosis as the first manifestation of systemic amyloidosis. Acta Med Croat 1992; 46: 245–50.
Guthoff R. Ultrasound in ophthalmologic diagnosis. New York: Thieme Medical Publishers, Inc., 1991.
Atta HR. Ophthalmic ultrasound. A practical guide. New York-EdinburghLondon-Madrid-Melbourne-San Francisco-Tokyo: Churchill Livingstone, 1996: 105–10.
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