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先天性血管畸形:边缘静脉和胚胎静脉的持续存在。

Congenital vascular malformations: the persistence of marginal and embryonal veins.

作者信息

Weber J, Daffinger N

机构信息

Diagnostische Angiologie und Inventions-Radiologie, Klinik Dr Guth, Hamburg, Germany.

出版信息

Vasa. 2006 May;35(2):67-77. doi: 10.1024/0301-1526.35.2.67.

DOI:10.1024/0301-1526.35.2.67
PMID:16796004
Abstract

BACKGROUND

In about 18% of cases with conginental vascular malformations we find a perspective of an atypical truncular vein, located along the outside of the leg, frequently extended from the dorsal foot up to the bottom. In presence of a normally developed system of the deep collecting veins of the lower limb and within the pelvic outflow we are talking about a persisting marginal vein (MV). Hypoplasia or even aplasia of the main deep veins in contrary defines the persisting embryonal vein (EV). Already in childhood these truncular dysplastic veins tend to develop varicose enlargement, causing severe reflux of a huge volume of blood--even more when being associated with av-fistulas (46%). In consequence a rapidly growing chronic venous insufficiency will guide to additional injuries.

PATIENTS AND RESULTS

We have analysed 97 patients showing a persisting MV (n: 92 ) within a total of 102 legs. A persistent embryonal vein (EV) was seen 10 times within this clientel. The persisting truncular veins, associated with phlebectasias and typical clinical symptoms have been examined in a diagnostic "step-by-step" procedure, mainly phlebographically (ascending leg phlebography and varicography), including direct venous blood pressure measurements (phlebodynamometry) and--if needed--by arteriography, showing av-shunting fistulae in 46% of cases. CT and MRI were consulted for the exact therapy planing (frequently initially offered as a non-invasive, however, inadequate key of diagnostic). Actually now these techniques cannot replace pre-operatively the angiographic imaging techniques.

CONCLUSIONS

The analysis of clinical, morphologic and functional signs, guiding to a specific therapy-relevant classification of MV's and EV's will be presented. And a specific strategy of surgical repair, interventional treatment of av-fistulas and conservative compressive follow-up treatment attempting palliative recompensation of the diseased venous outflow will be discussed also.

摘要

背景

在大约18%的先天性血管畸形病例中,我们发现一条非典型的主干静脉,位于腿部外侧,通常从足背延伸至足底。在下肢深静脉收集系统发育正常且盆腔静脉流出道正常的情况下,我们称之为持续存在的边缘静脉(MV)。相反,主要深静脉发育不全甚至缺如则定义为持续存在的胚胎静脉(EV)。这些主干发育异常的静脉在儿童时期就容易出现静脉曲张性扩张,导致大量血液严重反流——与动静脉瘘相关时更是如此(46%)。结果,快速进展的慢性静脉功能不全将导致更多损伤。

患者与结果

我们分析了97例患者,共102条腿,其中92条腿存在持续的MV。在这些患者中,有10次发现了持续存在的胚胎静脉(EV)。对伴有静脉扩张和典型临床症状的持续主干静脉进行了诊断性“逐步”检查,主要是静脉造影(下肢上行静脉造影和静脉造影),包括直接静脉血压测量(静脉测压法),必要时进行动脉造影,46%的病例显示有动静脉分流瘘。为制定精确的治疗方案参考了CT和MRI(通常最初作为非侵入性检查提供,但诊断价值不足)。实际上,目前这些技术无法在术前替代血管造影成像技术。

结论

将介绍对临床、形态学和功能体征的分析,这有助于对MV和EV进行与治疗相关的特定分类。还将讨论手术修复的具体策略、动静脉瘘的介入治疗以及保守压迫随访治疗,尝试对病变静脉流出道进行姑息性代偿。

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Congenital vascular malformations: the persistence of marginal and embryonal veins.先天性血管畸形:边缘静脉和胚胎静脉的持续存在。
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