A case of POEMS syndrome with cystoid macular edema

Abstract

To introduce a case of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome with bilateral cystoid macular edema (CME). Interventional case report. Temporal changes of serum vascular endothelial growth factor (VEGF) levels and macular thickness were evaluated. A 53-year-old woman with POEMS syndrome was referred complaining of long-standing impaired vision. Typical CME demonstrating petal-like dye pooling was observed in both eyes. After subtenon administrations of triamcinolone acetonide (TA), pars plana vitrectomy, and intraocular TA injection in the right eye, macular thickness decreased. Intraocular VEGF levels were <31 pg/ml at the vitrectomy and intraocular TA injection, whereas serum the VEGF level was >2000 pg/ml throughout the follow-up periods. In the untreated left eye, macular thickness altered related to serum VEGF level. Based on the low intraocular VEGF level and serum VEGF-related change of macular thickness, elevated serum VEGF might be a cause of CME in POEMS syndrome.