Optic Disc Edema, Cystoid Macular Edema, and Elevated Vascular Endothelial Growth Factor in a Patient With POEMS Syndrome

Abstract

A 48-year-old man with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome had bilateral optic disc edema (ODE), bilateral cystoid macular edema (CME), anasarca, and elevated serum vascular endothelial growth factor (VEGF). This is the first reported example of ophthalmoscopic, angiographic, and optical coherence tomographic evidence of the combination of ODE and CME in this syndrome. This combination of features suggests that the ODE in this condition may be due to increased vascular permeability.

Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: Evaluation and management

“…Visual field testing usually demonstrates enlargement of the blind spot, 125,291 although arcuate defects are also described. 24,52,94 FA typically shows late optic disk leakage and may also demonstrate petaloid leakage consistent with CME. OCT may also confirm the presence of CME or macular detachment.…”

“…64 The most common ocular finding in POEMS syndrome is bilateral optic disk edema (ODE), occurring in 29e73% of patients. 34,38,45,52 Kaushik et al, 125 in the largest study to date, found ODE in 17 of 33 patients (52%). Of note, five (29%) of the patients had asymptomatic ODE at the time of initial examination, leading the authors to advocate screening ophthalmologic examinations for all patients diagnosed with POEMS.…”

“…125 When presenting with symptomatic ODE, patients often complained of blurry vision, ocular pain, or diplopia. In addition to ODE, there are at least five cases with associated CME 52,94,110,290 and one of bilateral serous macular detachment. 209 Adjunctive testing can aid in the evaluation of these patients and augment physical examination findings.…”

A case of POEMS and chronic papilledema with preserved optic nerve function

“…7 Alternatively, the optic nerve swelling may be related to increased vascular permeability and serum vascular endothelial growth factor or cytokine levels, as seen in a few cases where patients presented concurrently with macular edema. 8,9 A recent retrospective case series suggests that papilledema may be an independent risk factor for POEMS-related mortality. 10 Therefore, POEMS should be kept on the differential for bilateral optic nerve swelling.…”

“…7 A retrospective case series looking at ocular findings in POEMS found that the most common visual field defects at initial ophthalmological assessment were bilaterally enlarged blind spots (75%, n ¼ 8), followed by general constriction (n ¼ 1), and nasal step defects (n ¼ 1). 8 Initial assessment showed good mean visual acuity, at 20/20 OD and 20/25 OS.…”