Periocular and Orbital Amyloidosis. Clinical Characteristics, Management, and Outcome

Leibovitch, I., Dinesh, S., Goldberg, R. A., Sullivan, T. J., Saeed, P., Davis, G., McCann, J. D., McNab, A. and Rootman, J. (2006) Periocular and Orbital Amyloidosis. Clinical Characteristics, Management, and Outcome. Ophthalmology, 113 9: 1657-1664. doi:10.1016/j.ophtha.2006.03.052


Author Leibovitch, I.
Dinesh, S.
Goldberg, R. A.
Sullivan, T. J.
Saeed, P.
Davis, G.
McCann, J. D.
McNab, A.
Rootman, J.
Title Periocular and Orbital Amyloidosis. Clinical Characteristics, Management, and Outcome
Journal name Ophthalmology   Check publisher's open access policy
ISSN 0161-6420
Publication date 2006
Sub-type Article (original research)
DOI 10.1016/j.ophtha.2006.03.052
Volume 113
Issue 9
Start page 1657
End page 1664
Total pages 8
Editor A. P. Schachat
Place of publication New York, U.S.A.
Publisher Elsevier
Collection year 2006
Language eng
Subject C1
730111 Hearing, vision, speech and their disorders
321016 Opthalmology and Vision Science
Abstract Objective: To present the clinical features and management outcome in a large series of patients with periocular and orbital amyloidosis. Design: Retrospective, noncomparative, interventional case series. Patients: All patients diagnosed with periocular and orbital amyloidosis in 6 oculoplastic and orbital units. Methods: Clinical records of all patients were reviewed. Main Outcome Measures: Clinical presentation, radiological and histological findings, treatment modalities, and outcome. Results. 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. Conclusion: 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.
Keyword Ophthalmology
Localized Conjunctival Amyloidosis
Systemic Amyloidosis
Surgical-management
Extraocular-muscle
Ptosis
Tumor
Q-Index Code C1

Document type: Journal Article
Sub-type: Article (original research)
Collections: 2007 Higher Education Research Data Collection
Institute for Molecular Bioscience - Publications
 
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Created: Wed, 15 Aug 2007, 10:57:05 EST