Periocular and orbital amyloidosis: clinical characteristics, management, and outcome.
OBJECTIVE
To present the clinical features and management outcome in a large series of patients with periocular and orbital amyloidosis.


METHODS
Retrospective, noncomparative, interventional case series.


METHODS
All patients diagnosed with periocular and orbital amyloidosis in 6 oculoplastic and orbital units.


METHODS
Clinical records of all patients were reviewed.


METHODS
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.


CONCLUSIONS
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.
Leibovitch
Igal
I
Oculoplastic & Orbital Division, Department of Ophthalmology & Visual Sciences, Royal Adelaide Hospital, University of Adelaide, Adelaide, Australia. leiboigal5@yahoo.com.au
Selva
Dinesh
D
Goldberg
Robert A
RA
Sullivan
Timothy J
TJ
Saeed
Peerooz
P
Davis
Garry
G
McCann
John D
JD
McNab
Alan
A
Rootman
Jack
J
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000175:diagnosis* / Q000628:therapy; D001763:Blepharoptosis / Q000175:diagnosis / Q000628:therapy; D003131:Combined Modality Therapy; D003229:Conjunctival Diseases / Q000175:diagnosis* / Q000628:therapy; D004172:Diplopia / Q000175:diagnosis / Q000628:therapy; D005094:Exophthalmos / Q000175:diagnosis / Q000628:therapy; D005260:Female; D006801:Humans; D007766:Lacrimal Apparatus Diseases / Q000175:diagnosis* / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015835:Ocular Motility Disorders / Q000175:diagnosis / Q000628:therapy; D013508:Ophthalmologic Surgical Procedures; D009916:Orbital Diseases / Q000175:diagnosis* / Q000628:therapy; D010146:Pain / Q000175:diagnosis; D011878:Radiotherapy; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome