Optical Coherence Tomography Features of POEMS Syndrome and Castleman Disease-associated Papillopathy

Abstract

Purpose: To report ocular symptoms and optical coherence tomography (OCT) features of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome.

Methods: Case report of two Caucasian patients with POEMS, one of whom also had multicentric Castleman disease.

Results: Both patients presented with edema on both optic disks, bilateral macular edema was seen in case 1, and peripapillary choroidal neovascularization was seen on the right side in case 2. OCT confirmed papilledema involving all retinal layers, with additional bilateral peripapillary serous retinal detachment involving the macula in both cases, and peripapillary CNV in case 2. Changes in retinal nerve fiber layer thickness, total peripapillary retinal thickness, and macular detachment were followed with OCT over the course of the disease and after administration of systemic therapy.

Conclusion: OCT examination of the optic nerve head and the macula has an important additional role in the diagnosis and follow-up of POEMS syndrome and Castleman disease.

• Castleman disease
• macular edema
• optic disk edema
• optical coherence tomography
• POEMS syndrome