To determine the clinical features, management and outcome of periocular and orbital amyloidosis in a tertiary UK service.

Retrospective, interventional, case series of patients treated at University Hospitals Leicester between 2004 and 2012. Casenotes of all patients diagnosed with amyloidosis were analysed. Clinical features, investigations, treatment modalities and outcomes were evaluated.

Casenotes of 5 patients (3 females and 2 males) were analysed. 3 patients presented with eyelid lesions, 2 with proptosis. The mean age at presentation was 60.6 +/- 5.1 yrs, the mean follow up was 36.6 months. 4 of 5 (80%) patients were managed in conjunction with the National Amyloidosis Centre.
4 of 5 patients had Lambda light chain disease. The mean number of imaging procedures was 2.2. 3 of 5 patients had systemic involvement; 4 of 5 patients did not have visceral involvement on SAP scintigraphy. Occult systemic disease was detected by PET-CT in 1 patient previously undetected by SAP scintigraphy.
4 of 5 patients underwent surgical resection, the mean number of operations was 1.6. 2 patients required multiple operations for progressive disease.
1 patient underwent treatment with systemic Infliximab but also required surgery for ocular disease.

Amyloidosis is a rare disorder which is often progressive and difficult to manage effectively. Surgical resection remains the mainstay of treatment for localised periocular disease. In our series, multidisciplinary management in conjunction with a specialist centre and newer imaging techniques have obviated the need for multiple invasive systemic biopsies. We advocate the use of PET-CT as a new imaging technique to detect occult systemic disease.