Carey Andrew Rising, Jeyaseelan Praveen
Neuro-Ophthalmology, Wilmer Eye Institute, 600 N Wolfe Street, Baltimore, MD, 21287, USA.
Clinical Neuro-Ophthalmology Fellow, Wilmer Eye Institute, 600 N Wolfe Street, Baltimore, MD, 21287, USA.
Int J Retina Vitreous. 2021 Jan 12;7(1):6. doi: 10.1186/s40942-020-00278-1.
POEMS (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.
A 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.
The 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.
POEMS（多发性神经病、脏器肿大、内分泌病、骨髓瘤蛋白、皮肤改变）综合征是一种罕见的累及多系统的血液疾病。病因不明。其特征为浆细胞、血小板及血管内皮生长因子（VEGF）水平升高。52%的患者会出现视盘水肿，这可能威胁视力，但视盘水肿的确切病因尚不确定。我们报告了一例POEMS综合征患者出现周边视网膜渗漏的罕见病例。
一名60岁患有POEMS综合征的女性出现双侧视力模糊。眼底检查显示双眼视盘水肿3级。腰椎穿刺显示初压正常。脑脊液分析显示蛋白升高但无细胞。脑部MRI及头部磁共振静脉血管造影未见异常。广角荧光素血管造影显示多灶性微小血管渗漏及颞前部明显渗漏。
作者推测POEMS综合征中的视盘水肿可能是由于VEGF（血管内皮生长因子）水平升高导致视盘处血管通透性增加所致。虽然视盘渗漏在眼底荧光素成像中是一个有充分记录的发现，但POEMS综合征中的周边视网膜渗漏尚未见报道。
