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

Ecsedy, Mónika; Schneider, Miklós; Nemes, J; Németh, János; Récsán, Zsuzsa

doi:10.3109/09273948.2014.883548

Cited by 13 publications

(11 citation statements)

References 24 publications

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“…II). We describe the ocular findings in a patient who fulfilled the criteria for POEMS syndrome and report bilateral papilledema and unilateral active choroidal neovascularization (CNV), a complication described only once previously (5).…”

Section: Introductionmentioning

confidence: 83%

Peripapillary Choroidal Neovascularization as an Unusual Complication of POEMS Syndrome

Nicolai

Cassiman

Delforge

et al. 2017

Ophthalmology @ Point of Care

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PATIENTS @ PoINT of cArE findings in a patient who fulfilled the criteria for POEMS syndrome and report bilateral papilledema and unilateral active choroidal neovascularization (CNV), a complication described only once previously (5). case presentation A 61-year-old man had a 1-year history of progressive demyelinating polyneuropathy and a monoclonal plasma cell proliferative disorder, responsible for loss of sensitivity in limbs, pain, weakness, and walking problems. Several weeks later, he developed malleolar edema, dermal angiomas, hepatomegaly, and splenomegaly. Based on the presence of the mandatory criteria as well as a number of other major and minor criteria, the suspected diagnosis of POEMS was confirmed (Tab. I), and treatment consisted of plasmapheresis (without result), lenalidomide 25 mg daily (Revlimid), and weekly dexamethasone 20 mg. One month later, in August 2016, the patient was referred to our department because of complaints of diplopia and metamorphopsia. On examination, the best-corrected visual acuity was 20/33 in the right eye (RE) and 20/67 in the left eye (LE). No abnormalities were noted in the anterior segment. Ocular examination revealed a left divergent strabismus. Ocular motility was normal as well as pupillary reaction. Funduscopic examination showed bilateral papilledema

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Section: Introductionmentioning

confidence: 83%

Peripapillary Choroidal Neovascularization as an Unusual Complication of POEMS Syndrome

Nicolai

Cassiman

Delforge

et al. 2017

Ophthalmology @ Point of Care

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“…Other ocular manifestations reported in the literature include cystoid macular edema [ 4 – 6 ], serous macular detachment [ 7 , 8 ], venous sinus thrombosis [ 9 ], infiltrative orbitopathy [ 10 ], uveitis [ 11 ], neovascularization of the disc [ 10 ], peripapillary choroidal neovascularization, and optic disc drusen [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Atypical central retinal artery occlusion as the first presentation of POEMS syndrome: a case report

et al. 2018

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BackgroundPOEMS syndrome is a plasma cell disorder, which clinically manifests from paraneoplastic syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. The most common ocular manifestation is optic disc swelling, whereas other ocular manifestations; cystoid macular edema, serous macular detachment, venous sinus thrombosis, infiltrative orbitopathy, uveitis, neovascularization of the disc, peripapillary choroidal neovascularization and optic disc drusen, had also been reported.Case presentationA 52-year-old Thai man presented with 5-day sudden painless visual loss in the left eye. Ocular examination revealed visual acuity of 20/20 and no light perception in the right and left eye, respectively. Right fundoscopic examination was significant for hyperemic generalized optic disc swelling. Left fundoscopic examination revealed opaque and edematous entire retina giving the appearance of central retinal artery occlusion (CRAO) along with pallid “chalky white” optic disc swelling. Fluorescein angiography showed profound leakage of bilateral optic nerve heads and arteriolar filling defect in macular area along with leakage of small retinal arterioles in the left eye. Indocyanine green angiography demonstrated choroidal filling defect in the left eye only. Neuroimaging showed enhancement and luminal narrowing of left internal carotid artery, early subacute watershed infarctions in the left cerebral hemisphere and pachymeningeal enhancement. Cerebrospinal fluid analysis revealed high protein level with normal opening pressure. Intravenous methylprednisolone was initially started without any benefit. After extensive investigations, diagnosis of “POEMS syndrome” was made based on polyneuropathy, elevated lambda light chain level, elevated plasma vascular endothelial growth factor (VEGF), hepatomegaly, spinal sclerotic bone lesions, and thrombocytosis. Furthermore, sural nerve biopsy demonstrated neuropathy and positive VEGF staining. He was treated with eight cycles of bortezomib, cyclophosphamide and dexamethasone (BorCyDex). Polyneuropathy and thrombocytosis had remarkably improved after 2nd cycle, whereas, visual impairment had shown no recovery. Hepatomegaly was significantly reduced after the completion of BorCyDex. Our case eventually received autologous hematopoietic stem cell transplantation with high dose melphalan.ConclusionsTo our knowledge, we illustrated the first patient given CRAO as the first presentation and ocular finding ever reported in POEMS syndrome. Both cerebral and ocular infarctions were presumably the result of VEGF-induced cranial vasculopathy as evidenced by neuroimaging.

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“…Unfortunately, they did not observe an improvement of the ODE. 9 The purpose of this report is to present our findings in a patient with the POEMS syndrome who was successfully treated with bortezomib and dexamethasone followed by autologous peripheral blood stem cell transplantation. We followed the reduction of the ODE objectively and quantitatively by measuring the changes of the pRT by SD-OCT before and after the treatments.…”

Section: Introductionmentioning

confidence: 99%

Reduction of Optic Disc Oedema by Bortezomib and Dexamethasone Followed by Autologous Peripheral Blood Stem Cell Transplantation in Patient with POEMS Syndrome

Iwase

Yokouchi

Misawa

et al. 2017

Neuro-Ophthalmology

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The authors present findings in a 39-year-old man with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome with bilateral optic disc oedema. He was successfully treated with bortezomib and dexamethasone followed by autologous peripheral blood stem cell transplantation. The peripapillary retinal thickness was reduced in the optical coherence tomographic (OCT) images along with a decrease of the serum vascular endothelial growth factor (VEGF) levels. The authors recommend OCT to monitor the changes in the signs of POEMS syndrome after treatments.

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

Abstract: 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.

Cited by 13 publications

References 24 publications

Peripapillary Choroidal Neovascularization as an Unusual Complication of POEMS Syndrome

Peripapillary Choroidal Neovascularization as an Unusual Complication of POEMS Syndrome

Atypical central retinal artery occlusion as the first presentation of POEMS syndrome: a case report

Reduction of Optic Disc Oedema by Bortezomib and Dexamethasone Followed by Autologous Peripheral Blood Stem Cell Transplantation in Patient with POEMS Syndrome

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