Local Treatment with Triamcinolone Acetonide and Bevacizumab for Ocular Symptoms in a Patient with POEMS Syndrome

Abstract

Purpose: To report our experience in managing a case of bilateral optic disc oedema and unilateral cystoid macular oedema with a posterior subtenon (PST) injection of triamcinolone acetonide and intravitreal bevacizumab (IVB) in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome.

Case report: A 50-year-old healthy woman complaining of bilateral blurred vision for several days was referred to the ophthalmologist. An eye examination showed a bilateral optic disc swelling and small preretinal haemorrhages. In addition, a peripheral neuropathy was found. Laboratory tests showed elevated levels of M-protein type IgA of 1.4 g/l and a vascular endothelial growth factor (VEGF) concentration of 6.1 ng/ml. The cerebrospinal fluid protein content was 131 mg/dl. An abdominal CT scan revealed hepatosplenomegaly. The diagnosis of POEMS syndrome was made based on the polyneuropathy, hepatosplenomegaly, IgA lambda monoclonal proteinemia, and optic disc swelling. As vision declined, 40 mg PST injection of triamcinolone was given bilaterally. Ten weeks thereafter, 2 IVB injections of 2.5 mg were administered to the left eye, with a 4-week interval. Because of an incomplete recovery of her visual functions, diarrhoea and general malaise, melphalan, followed by autologous peripheral blood stem cell transplantation, was initiated. After systemic treatment, she had no visual complaints anymore, the optic disc oedema had resolved, and the VEGF serum concentration had normalized.

Discussion: Local treatment with IVB and PST injection of triamcinolone can be an option for ocular symptoms in POEMS syndrome. However, given the incomplete recovery of the ocular abnormalities and the other symptoms, systemic treatment remains standard.

Keywords: Bevacizumab; Macular oedema; Optic disc oedema; POEMS syndrome; Triamcinolone.

Fig. 1

A fundus photograph of the right (a) and the left eye (b) with bilateral optic disc swelling. An ophthalmologic echography of the right (c) and the left eye (f): sagittal section of the optic nerve with visible disc oedema and small enlargement of the subdural space in the left eye (white arrows). The visual field of the right (d) and the left eye (e) was performed with Humphrey visual field using a 30-2 algorithm with SITA fast strategy. It shows abnormally enlarged blind spots and inferonasal deficits in both eyes.

Fig. 2

A macular thickness map with OCT image of the macula of the right (a, b) and the left eye (c, d) before treatment. Illustrations b and d show a cystoid fluid accumulation in the outer plexiform layer in the peripapillary area, and a subretinal fluid accumulation under the fovea (white arrows). The macular thickness map with OCT image of the macula of the right (e, f) and the left eye (g, h) after PST treatment. Illustrations f and h show a decrease in cystoid fluid accumulation in the outer plexiform layer in the peripapillary area as well as the subretinal fluid accumulation under the fovea.

Fig. 3

A macular thickness map with OCT image of the macula of the right (a, b) and the left eye (c, d) after the local IVB treatment. Illustrations b and d show further resolving of the cystoid fluid accumulation in the retina and no macular oedema. The macular thickness map with OCT images of the macula of the right (e, f) and the left eye (g, h) after local and systemic treatment. Illustrations f and h show a stable central foveal thickness. A fundus photograph of the right (i) and the left eye (j) without optic disc swelling.