Abstract
BackgroundPOEMS (polyneuropathy, organomegaly, endocrinopathy, myeloma protein, skin changes) syndrome is a rare blood disorder with multi-system involvement. The cause is unknown. It is marked by elevated plasma cells, platelets, & VEGF (vascular endothelial growth factor) levels. 52% of patients develop optic disc edema which may be vision threatening but the exact etiology of optic disc edema is uncertain. We report a rare finding of peripheral retinal leakage in POEMS syndrome.Case presentationA 60 year-old female with POEMS syndrome presented with bilateral blurred vision. Fundi showed grade 3 disc edema OU. Lumbar puncture showed normal opening pressure. CSF analysis showed elevated proteins with no cells. MRI brain and MR Venogram head were unremarkable. Wide field fluorescein angiography demonstrated multifocal tiny vascular leakage and significant anterior temporal leakage.ConclusionThe authors hypothesize the disc edema in POEMS syndrome may be caused by increased vascular permeability at the optic disc secondary to increased VEGF (vascular endothelial growth factor) levels. Though disc leakage is a well-documented finding in fundus fluorescein imaging, peripheral retinal leakage in POEMS syndrome is not reported.
Highlights
POEMS syndrome is a rare blood disorder with multi-system involvement
The authors hypothesize the disc edema in POEMS syndrome may be caused by increased vascular permeability at the optic disc secondary to increased Vascular endothelial growth factor (VEGF) levels
Though disc leakage is a well-documented finding in fundus fluorescein imaging, peripheral retinal leakage in POEMS syndrome is not reported
Summary
POEMS (polyneuropathy, organomegaly, endocrinopathy, myeloma protein, skin changes) syndrome is a rare blood disorder with multi-system involvement. It is marked by elevated plasma cells, platelets, & VEGF (vascular endothelial growth factor) levels. 52% of patients develop optic disc edema which may be vision threatening but the exact etiology of optic disc edema is uncertain. We report a rare finding of peripheral retinal leakage in POEMS syndrome. Case presentation: A 60 year-old female with POEMS syndrome presented with bilateral blurred vision. Fundi showed grade 3 disc edema OU. CSF analysis showed elevated proteins with no cells. MRI brain and MR Venogram head were unremarkable. Wide field fluorescein angiography demonstrated multifocal tiny vascular leakage and significant anterior temporal leakage
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