• Igal Leibovitch, Dinesh Selva, Robert A Goldberg, Timothy J Sullivan, Peerooz Saeed, Garry Davis, John D McCann, Alan McNab, and Jack Rootman.
• Oculoplastic & Orbital Division, Department of Ophthalmology & Visual Sciences, Royal Adelaide Hospital, University of Adelaide, Adelaide, Australia. leiboigal5@yahoo.com.au
• Ophthalmology. 2006 Sep 1;113(9):1657-64.

ObjectiveTo present the clinical features and management outcome in a large series of patients with periocular and orbital amyloidosis.DesignRetrospective, noncomparative, interventional case series.PatientsAll patients diagnosed with periocular and orbital amyloidosis in 6 oculoplastic and orbital units.MethodsClinical records of all patients were reviewed.Main Outcome MeasuresClinical presentation, radiological and histological findings, treatment modalities, and outcome.ResultsThe 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.ConclusionPeriocular 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.

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