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Type: Journal article
Title: Periocular and orbital amyloidosis - Clinical characteristics, management, and outcome
Author: Leibovitch, I.
Selva-Nayagam, D.
Goldberg, R.
Sullivan, T.
Saeed, P.
Davis, G.
McCann, J.
McNab, A.
Rootman, J.
Citation: Ophthalmology, 2006; 113(9):1657-1664
Publisher: Elsevier Science Inc
Issue Date: 2006
ISSN: 0161-6420
Abstract: <h4>Objective</h4>To present the clinical features and management outcome in a large series of patients with periocular and orbital amyloidosis.<h4>Design</h4>Retrospective, noncomparative, interventional case series.<h4>Patients</h4>All patients diagnosed with periocular and orbital amyloidosis in 6 oculoplastic and orbital units.<h4>Methods</h4>Clinical records of all patients were reviewed.<h4>Main outcome measures</h4>Clinical presentation, radiological and histological findings, treatment modalities, and outcome.<h4>Results</h4>The study included 24 patients (15 female, 9 male) with a mean age of 57+/-17 years. Nineteen cases were unilateral, and 5 were bilateral. Clinical signs and symptoms included a visible or palpable periocular mass or tissue infiltration (95.8%), ptosis (54.2%), periocular discomfort or pain (25%), proptosis or globe displacement (21%), limitations in ocular motility (16.7%), recurrent periocular subcutaneous hemorrhages (12.5%), and diplopia (8.3%). Seven cases had orbital involvement, and 17 were periocular. Immunohistochemistry in 7 patients showed B cells or plasma cells producing monoclonal immunoglobulin chains that were deposited as amyloid light chains. Only 1 patient was diagnosed with systemic amyloid light chain amyloidosis. Treatment modalities were mainly observation and surgical debulking. During a mean follow-up period of 39 months, 21% showed significant progression after treatment, whereas 79% were stable or showed no recurrence after treatment.<h4>Conclusion</h4>Periocular and orbital amyloidosis may present with a wide spectrum of clinical findings and result in significant ocular morbidity. Complete surgical excision is not feasible in many cases, and the goal of treatment is to preserve function and to prevent sight-threatening complications.
Keywords: Humans
Ocular Motility Disorders
Conjunctival Diseases
Lacrimal Apparatus Diseases
Orbital Diseases
Tomography, X-Ray Computed
Magnetic Resonance Imaging
Treatment Outcome
Combined Modality Therapy
Ophthalmologic Surgical Procedures
Retrospective Studies
Aged, 80 and over
Middle Aged
DOI: 10.1016/j.ophtha.2006.03.052
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