METHODS: We presented a dynamic evolution process of the IJVS formation, through a case of a 17-year-old female with paroxysmal nocturnal hemoglobinuria (PNH)-associated right internal jugular venous thrombosis (IJVT), which resulted in post-thrombotic IJVS in the rare context of rapid recanalization. Meanwhile, we compared her images with images of a 39-year-old healthy male with hypoplastic IJV to determine the differences between the acquired IJVS and congenital dysplasia.
RESULTS: Based on the first case, we noticed the whole formative process of acquired IJVS from nothing to something. Meantime, we found that acquired IJVS was surrounded by abnormal corkscrew collaterals as imaged on contrast-enhanced magnetic resonance venography (CE-MRV), and the ipsilateral jugular foramen (JF) was normal-sized as displayed on computer tomography (CT). Conversely, jugular hypoplasia was with ipsilateral stenotic JF and without serpentine collaterals.
CONCLUSIONS: JF morphology and venous collaterals may be deemed as surrogate identifiers to distinguish acquired unilateral IJVS from jugular hypoplasia.
方法:我们介绍了IJVS形成的动态演化过程,通过一例17岁女性阵发性夜间血红蛋白尿症(PNH)相关的右颈内静脉血栓形成(IJVT),在罕见的快速再通情况下导致血栓后IJVS。同时,我们将她的图像与一名39岁健康男性IJV发育不全的图像进行了比较,以确定获得性IJVS和先天性发育不良之间的差异.
结果:根据第一种情况,我们注意到获得IJVS的整个形成过程从无到有。同时,我们发现获得的IJVS被异常的开瓶器络脉所包围,如在对比增强磁共振静脉造影(CE-MRV)成像,计算机断层扫描(CT)显示的同侧颈静脉孔(JF)大小正常。相反,颈静脉发育不全伴同侧狭窄JF,无蛇纹石络脉。
结论:JF形态学和静脉侧支可被视为区分获得性单侧IJVS和颈静脉发育不全的替代标识符。