Optic disk drusen, peripapillary choroidal neovascularization, and POEMS syndrome

Diduszyn, Jorge M; Quillen, David A.; Cantore, William A.; Gardner, Thomas W.

doi:10.1016/s0002-9394(01)01270-3

Cited by 21 publications

(9 citation statements)

References 5 publications

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“…Vision is usually normal at onset, unless there are some underlying complications, such as cystoid macular edema and serous macular detachment. 10,11 Long-standing ODE can lead to optic nerve function deterioration, as in the case of this patient. Fourier domain optical coherence tomography on follow-up in the second year showed that ganglion cell complex thicknesses were reduced (Fig.…”

mentioning

confidence: 78%

Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome

Dong

Yin

White³

et al. 2018

Canadian Journal of Ophthalmology

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POEMS syndrome is a rare complex multisystem disorder of which typical features are polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS). The pathogenesis of this syndrome is not well understood. In recent years, it has been recognized as a paraneoplastic syndrome caused by an underlying plasma cell neoplasm. 1,2 Because its symptoms are complex and can mimic those of other disorders, it is difficult to diagnose. 3-6 POEMS syndrome has a rapidly progressive course, so making an early, accurate diagnosis can contribute to higher possibilities of survival. Optic disk edema (ODE) is an early and common ocular manifestation of POEMS syndrome, but being asymptomatic, it is likely to be misdiagnosed. Here, a case of POEMS syndrome that presented as ODE and juxtapapillary subretinal hemorrhage is described, and the potential diagnostic problems will be discussed to raise awareness of this disease to ophthalmologists. A 45-year-old female had experienced numbness and weakness in the lower limbs for 3 months, coinciding with weight loss (15 kg in 3 months), and blurred vision with metamorphopsia predominantly in her left eye for 1 week. The patient's blood pressure was 134/90 mm Hg, and body

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mentioning

confidence: 78%

Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome

Dong

Yin

White³

et al. 2018

Canadian Journal of Ophthalmology

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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%

“…POEMS syndrome is a plasma cell disorder, which clinically manifests from paraneoplastic syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes [ 1 ]. The most common ocular manifestation is optic disc swelling, whereas other ocular manifestations; cystoid macular edema (CME), serous macular detachment, venous sinus thrombosis, infiltrative orbitopathy, uveitis, neovascularization of the disc (NVD), peripapillary choroidal neovascularization and optic disc drusen, had also been reported [ 2 – 12 ]. To our knowledge, central retinal artery occlusion has never been reported in POEMS syndrome.…”

Section: Introductionmentioning

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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“…Ophthalmic complications in MCD are very rare with most published cases describing intraorbital and optic nerve lesions 2,3. Intraocular involvement is rarer with one published adult case of uveitis,4 two cases of bilateral,5,6 one case of unilateral serous retinal detachment and choroidal effusion,7 one case of hypertensive choroidopathy,8 one case of choroidal infiltrates,7 and one case of optic nerve drusen and peripapillary choroidal neovascularization in a patient with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome and associated Castleman’s disease 9. One pediatric case describing venous engorgement and tortuosity, intraretinal hemorrhages, cotton wool spots, and neurosensory detachment of the posterior pole was thought to be due to hyperviscosity syndrome which responded to one session of plasmapheresis 10.…”

Section: Discussionmentioning

confidence: 99%

Retinal vein occlusion during flare of multicentric Castleman's disease

Kozák

Reid

2013

OPTH

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Optic disk drusen, peripapillary choroidal neovascularization, and POEMS syndrome

Cited by 21 publications

References 5 publications

Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome

Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome

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

Retinal vein occlusion during flare of multicentric Castleman's disease

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Optic disk drusen, peripapillary choroidal neovascularization, and POEMS syndrome

Cited by 21 publications

References 5 publications

Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome

Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome

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

Retinal vein occlusion during flare of multicentric Castleman&#39;s disease

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Retinal vein occlusion during flare of multicentric Castleman's disease