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李明省 三甲
李明省 主治医师
郑大一附院 血管外科

我们应该如何治疗动静脉畸形(技巧和绝招)?(I)

How Do We Treat Arteriovenous Malformations (Tips and Tricks)?

我们应该如何治疗动静脉畸形(技巧和绝招)郑州大学第一附属医院血管外科李明省

Young Soo Do, MD, Kwang-Bo Park, MD, and Sung Ki Cho, MD

翻译:李明省(郑州大学第一附属医院神经介入科)

Ethanol embolization is sufficient to eliminate or improve symptoms of arteriovenous malformations (AVMs) in a high percentage of patients, but with substantial risk of minor and major complications. Inadvertent embolization must be avoided by superselective catheterization or direct puncture of the nidus. According to the angiographic morphology of the nidus, AVMs of the trunk and extremities can be classified into four types: type I (arterio-venous fistulae), type II (arteriolo-venous fistulae), type IIIa (arteriolo-venulous fistulae without dilation of the fistula), and type IIIb (arteriolo-venulous fistulae with dilation of the fistula). The described angiographic classification provides considerable information concerning the characteristics of AVMs in the body and extremities, the optimal therapeutic approach, and the likely therapeutic outcome. Tech Vasc Interventional Rad 10:291-298 © 2007 Elsevier Inc. All rights reserved. KEYWORDS: arteriovenous malformation, ethanol, embolization, angiography

无水乙醇栓塞能够有效消除或减轻大部分动静脉畸形患者的症状,但是或多或少都有一些并发症的发生。超选择栓塞和直接经皮穿刺畸形血管巢应该取代非超选择供养动脉无水乙醇注射,根据血管造影的形态学躯干和肢体的动静脉畸形可以分为四个类型:I型、主干动静脉瘘,II型、小动脉-静脉瘘,IIIa型、小动脉-小静脉瘘(不伴随瘘口扩张),IIIb、小动脉-小静脉瘘(伴随瘘口扩张),基于血管造影的分类系统能够为躯干和肢体血管畸形的治疗方法的选择和可能的预后判断提供相当多的参考信息。

【关键词】动静脉畸形,无水乙醇,栓塞,血管造影

Congenital arteriovenous malformations (AVMs) are a major challenge in medical practice, which are often associated with serious symptoms such as heart failure, neuropathy, pain, and bleeding. Embolotherapy has been a primary mode of treatment for the management of AVMs with improvements in catheter systems and selective techniques, which can be achieved through the transarterial, transvenous, or direct puncture approaches.

先天性动静脉畸形在临床中极具挑战,常常伴随心衰、神经病变、疼痛和出血,随着导管系统的改进和入路的多样化,硬化治疗已经成为动静脉畸形的主要治疗模式,我们能够通过动脉、静脉、甚至经皮穿刺到达病灶进行硬化栓塞治疗。

Despite the importance of angiography, most reports have traditionally described the angiographic features of AVMs in terms of feeding arteries, the complex network of arteriovenous shunts (referred to as the nidus), and draining veins. However, AVMs are so diverse and diffuse in nature that such descriptions are too broad to accurately represent the individual

characteristics of an AVM. Furthermore, such descriptions have limitations in terms of aiding therapeutic decision making by interventional radiologists and in terms of the exchange of angiographic data between therapeutic teams.

血管造影是血管畸形分类的极为重要依据,大多数文献仍从供养动脉、瘤巢和引流静脉三个方面情况来描述动静脉畸形。然而动静脉畸形在本质上又千差万别,现有的分类系统太过于宽泛,不能够精准地描述所有个例的特点。此外,这种分类系统在放射介入医生制定治疗计划和造影数据交流方面存在局限。

Recently, to help the therapeutic decision making for performing embolotherapy, we have proposed four prototypes of peripheral AVMs based on the angiographic morphology of the nidus. In this article, we described therapeutic results and approaches to the ethanol embolization of AVMs according to a newly proposed angiographic classification of AVMs of the body and extremities.

近来,为辅助栓塞治疗方案的制定,基于瘤巢血管造影形态学我们提出4种类型的分类方法原型,本文里我们将基于新的分类系统介绍无水乙醇栓塞治疗躯干和肢体动静脉畸形的目标和结果。

Techniques and Methods Patient Selection Criteria All patients referred to our hospital had serious problems. These included severe pain, a pulsating growing mass, ulceration at the lesion site, bleeding at the ulcerated skin, vaginal bleeding, dysmenorrhea, bone overgrowth at the lesion site, symptoms or signs of congestive heart failure consisting of dyspnea on exertion and cardiomegaly, ischemic symptoms at the extremities of the distal part of AVMs as a result of a steal effect through a high-flow shunt, or their combinations. The patients usually had more than one symptom and sign. Except severe infection associated with AVMs, the AVMs of extremity and trunk were treated by ethanol embolization.

技术和方法

患者选择标准

所有入选患者均有严重临床症状,比如:严重的疼痛、搏动性生长的肿块、病变部位溃疡、溃疡皮肤的出血、阴道出血、痛经、病变部位骨过度生长,劳力性呼吸困难、心脏肥大等充血性心力衰竭症状,以及因AVM分流所致的远端肢体缺血症状或其合并症。入选患者常常不止出现一种症状,除AVM合并感染外,所有肢体和躯干AVM均接受无水乙醇硬化栓塞治疗。

Diagnosis and Follow-up of AVMs The diagnoses of AVMs were made based on the clinical and imaging examinations. The lesion was first assessed by a physical examination. Multidetector computed tomography (MDCT) or magnetic resonance (MR) imaging besides the selective angiography should be performed to obtain the detailed anatomical and hemodynamic information of AVMs. MDCT or MR imaging are also good imaging modalities for the evaluation of residual lesions or recurrence after treatment.

AVM的诊断和随访

AVM需基于临床表现和影像检查综合得出诊断结论,影像检查之前需要进行详细的体格检查,血管造影之前需要完善多层螺旋CT检查或磁共振检查,以便了解畸形血管团的解剖和血流动力学信息,多层螺旋CT或磁共振检查还是评价残余病灶和复发病灶的良好手段。

The Angiographic Classification of AVMs It is well known that the nidus of AVMs is fundamentally a conglomeration of arteriovenous fistulae. AVMs of the body and extremities can be classified into four types according to their angiographic morphologies (Fig. 1), as follows: type I (arterio-venous fistulae), when at most three separate arteries are shunted to a single draining vein; type II (arteriolo-venous fistulae), when multiple arterioles are shunted into a single draining vein; type III (arteriolo-venulous fistulae), when multiple shunts are located between the arterioles and venules. In this type, if the fistula unit of the nidus is observed as a blush or fine striation on angiography, it is subdivided into either; type IIIa (arteriolo-venulous fistulae with nondilated fistula), when the fistula unit of the nidus is observed as a complex vascular network; or type IIIb (arteriolo-venulous fistulae with dilated fistula). This classification can be regarded as a modification of the previous classification proposed by Houdart and coworkers, because they classified intracranial AVMs into three types based on the morphology of the nidus—arterio-venous, arteriolo-venulous, and arteriolo-venulous fistulae.

AVM的血管造影分类方法

从根本上讲,动静脉畸形的瘤巢实际上是包含小动脉-小静脉瘘的团块,根据血管造影形态学躯干和肢体AVM可以分成以下4种类型:I型、主干动静脉瘘(不超过3根单独的动脉分流到一根引流静脉);II型、小动脉-静脉瘘(多支<< span="">超过3支>小动脉分流到单支引流静脉);III型、小动脉-小静脉瘘,根据造影表现为“肿瘤”染色还是“线样征”又可再分为IIIa型和IIIb型,其中IIIa型、小动脉-小静脉瘘不伴随瘘口扩张,也就是瘤巢的瘘表现为复杂的血管网,IIIb型、小动脉-小静脉瘘伴随瘘口扩张。Houdart及其同事基于形态学将颅内AVM分为瘤巢-动脉-静脉,小动脉-小静脉和小动脉-小静脉瘘,本分类方法可以看作是对Houdart及其同事分类方法的改进。

1.

Type I (arterio-venous fistulae) AVMs: No more than three separate arteries shunt to the initial part of a single venous component.

I型、主干型动静脉瘘:不超过3根单独动脉分流至单根静脉的起始部

Type II (arteriolo-venous fistulae): Multiple arterioles shunt to the initial part of a single venous component, in which the arterial components show a plexiform appearance on angiography.  

II型、小动脉-静脉瘘:超过3根以上动脉瘘到单根静脉起始部,造影表现为网丛状的染色

Type IIIa (arteriolo-venulous fistulae with nondilated fistula): Fine multiple shunts are present between arterioles and venules and appear as a blush or fine striation on angiography.

IIIa型、不伴随瘘口扩张的小动脉-小静脉瘘,小动脉-小静脉之间多发细小瘘,造影表现为“肿瘤”染色或细条纹状血流

Type IIIb (arteriolo-venulous fistulae with dilated fistula): Multiple shunts are present between arterioles and venules and appear as a complex vascular network on angiography.

IIIb型、伴随瘘口扩张的小动脉-小静脉瘘:多发的小动脉和小静脉之间的直接分流,血管造影表现为复杂的的血管网络

Procedures All of the ethanol embolization was performed under general anesthesia to control the pain, and Swan-Ganz and arterialline monitoring were performed in most patients to monitor the pulmonary artery pressure and arterial blood pressure. The objective of the staged ethanol embolization was to embolize not vascular feeders but all or part of the nidus until the complete resolution or improvement in the clinical symptoms and signs was achieved. In every session of embolotherapy, baseline selective and superselective angiographic studies were performed to determine the exact flow characteristics of the AVMs. After considering not only the angiographic features of the AVMs but also other factors such as important normal arterial or venous branches arising in very close proximity to a malformation and extreme arterial tortuosity or previous surgery precluding a successful transarterial catheterization, the approach of vascular access to attack the nidus itself was chosen. Transarterial, transvenous catheterization using a coaxial catheter, and/or percutaneous direct puncture was needed to reach the nidus being embolized. When focusing only on the morphology of the nidus, the type I and II AVMs can be embolized through any of the three approaches: transarterial, transvenous, or direct puncture approaches. The type IIIa AVMs can be treated only through a transarterial approach because they are too fine to be punctured directly. The type IIIb AVMs can be treated through either the transarterial or direct puncture approaches. In the type III AVMs, embolization through the transvenous approach is contraindicated because embolic materials introduced by a transvenous approach will not reach the shunts but block the venous drainage. Therefore, this will result in hypertension in the shunts and aggravation of AVMs if the arterial contribution is not disrupted. By the definition of this classification, the type II AVMs have multiple tortuous feeding arterioles and a large dilated venous component. Therefore, it is important to attack the venous component of the nidus through a direct puncture or transvenous approaches rather than through the transarterial approach. Also, a direct puncture and transvenous approach can avoid the risk of an inadvertent occlusion of the adjacent normal vessels by the transarterial approach. In the mixed type AVMs, the transarterial and direct puncture approaches of embolotherapy, which can treat both components of the AVMs simultaneously, are preferred.

操作步骤所有无水乙醇硬化栓塞均在全麻下进行以控制患者术中疼痛,术中应用Swan-Ganz气囊漂浮导管监测肺动脉压,应用有创动脉压监测装置监测血压。分次的、有计划的无水乙醇硬化栓塞目标不是消除病变的供养动脉而是部分甚至全部的瘤巢直至临床症状和体征改善直至完全消失。每一个栓塞治疗前都要进行选择性和超选择性血管造影以便了解病灶的血流动力学特点,除了考虑AVM的造影特点,还要考虑治疗时瘤巢附近的正常小动脉或小静脉可能受到的影响、入路血管迂曲情况、之前外科手术对插管带来的影响等,才最终选择能到达瘤巢的入路血管实施硬化栓塞治疗。樱花治疗AVM的瘤巢可以使用同轴导管从动脉、静脉入路,也可以经皮穿刺直达瘤巢进行,对于Ⅰ、Ⅱ型AVM若仅考虑瘤巢以上三种入路均可到达病灶进行治疗。对于IIIa型因病变血管太细无法经皮穿刺,只能经动脉途径治疗,IIIb型经动脉途径和经皮穿刺途径均可。对于IIIAVM禁忌从静脉入路进行硬化栓塞,因为这样既不能让栓塞材料到达瘘口,还会导致栓塞材料阻塞静脉流出道。因为这样会导致分流道压力升高,同时供养动脉违背破坏掉会导致畸形血管团的进展。从这个分类方法的定义也可以知道,IIAVM有多个迂曲的供养小动脉同时还有一个大的扩张的静脉成分,因此对于该型AVM的治疗更重要的是从静脉入路或经皮穿刺直接到达瘤巢,而不是从供养动脉硬化栓塞。经皮穿刺或经静脉入路可以避免动脉非超选择栓塞带来的邻近正常结构的异位栓塞。对于混合类型的AVM,经动脉和经皮穿刺途径联合应用首选,可以同时治疗AVM的两种成分。

To determine the volume of ethanol used during embolizations and the rate of injection, test injections of the contrast medium were performed under fluoroscopic monitoring. The amount of ethanol used was based on the amount of contrast medium required to fill the AVM nidus without opacifying normal vessels. When maximum amounts of ethanol were used, the total dose did not exceed 1 mL/kg of body weight. In some cases, to achieve vascular stasis, a proximal inflow occlusion was performed during the ethanol injection using either an intravascular occlusion balloon catheter for the trunk and pelvis area or external pneumatic blood pressure cuffs for extremities. Of the embolic materials, absolute ethanol (99%) was used in most cases and 50-70% ethanol with a nonionic contrast medium (Xenetix 300; Guerbet, Cedex, France) was used in some AVMs of the hands or feet. Additional coil embolizations (Nester coil; Cook, Bloomington, IN) of the dominant outflow vein were performed in Type II AVMs by direct puncture or transvenous catheterization to reduce the amount of ethanol and to stabilize the thrombosis in the dominant outflow vein as Yee and Yakes previously reported (Fig. 2). After waiting 5 to 10 minutes after the ethanol injection, an arteriogram was performed to determine whether AVMs had been embolized completely. Complete embolization of at least one compartment of the AVMs required the meticulous repetition of the technique described earlier. When an elevated pulmonary artery pressure (more than 25 mm Hg) was sustained at the end of the session or a large amount of absolute ethanol was injected, the patient was kept at the intensive care unit for close pulmonary artery pressure monitoring and continuous administration of nitroglycerine (0.3-3.0 µg/kg/min). All patients were closely followed up to identify any immediate or delayed complications.

为了预判无水乙醇硬化栓塞时的注射速率,可以使用造影剂在透视下进行实验性注射,无水乙醇的用量随造影剂的用量而定,以不栓塞正常血管为宜,无水乙醇最大用量不超过体重(kgmL,在某些情况下为了达到血管内栓塞剂滞留可以在躯干和盆腔血管内使用球囊阻断,也可以在肢体外使用血压计充气袖带加压法阻断静脉回流。栓塞材料选择上,99%无水乙醇使用最为广泛,但栓塞手、足AVM50-70%酒精和非离子碘造影剂配比后应用较多。如YeeYakes之前所报道的一样,对于IIAVM经静脉或经皮穿刺病灶使用弹簧圈栓塞流出道可以减少无水乙醇用量,同时也可以使栓子形成更加稳固。无水乙醇栓塞5~10分钟后复查动脉造影了解病灶是否完全消失,完全治愈AVM需要将前述技术精细地反复执行。当治疗临近尾声肺动脉压超过25mmHg或大剂量无水乙醇注射后,患者需要在重症监护室监测肺动脉压并持续以0.3-3.0 µg/kg/min泵入硝酸甘油,所有患者都需密切随访以防术后即刻并发症或延迟出现的并发症。

As a rule, additional embolization was recommended if the symptoms and signs remained or the AVMs remained at follow-up imaging studies. The recommended time interval between embolotherapy is more than 2 months.

作为一个原则,当症状没有改善或影像随访证实病灶未消失,建议再次硬化栓塞,两次治疗的间隔建议超过2个月。

A diagrammatic presentation of approaches to ethanol embolotherapy according to the angiographic type.

根据血管造影分类方法的硬化栓塞术入路示意图:

2.

(A) The main target of type II AVMs is the venous component of the nidus. Therefore, the mainstay therapeutic approaches are transvenous (TV) and direct puncture (DP). Before ethanol embolization, coil embolization of the venous component of the nidus through a transvenous or direct puncture approach is often required to reduce the amount of ethanol and to stabilize the thrombosis within the large venous component.

IIAVM主要病灶是瘤巢的静脉成分,因此主要的治疗入路是经静脉途径和直接经皮穿刺,在无水乙醇硬化治疗之前,经静脉途径或经皮穿刺使用弹簧圈栓塞病灶的静脉成分以减少无水乙醇用量,也能够阻塞流出道进而使栓子形成更加稳固。

(B) Type IIIa AVMs. Only the transarterial (TA) approach is available. Because the fistula is too fine, direct puncture of the AVMs is not possible.

对于IIIaAVM只能经过动脉进行治疗,因瘘口过于细小,直接穿刺无法实现。

(C)Type IIIb AVMs. These AVMs can be treated properly via transarterial (TA) and direct puncture (DP) approaches. However, if there are no obstacles in terms of access and safe embolization, the transarterial approach is preferred because of familiarity with arterial angiographic findings, the ease of detecting a normal artery arising near an AVM, and the direct puncture hazard whereby ethanol can leak into adjacent soft tissues. The treatment of type III AVMs via a transvenous approach is contraindicated. The approaches used for mixed types are combinations of those used to treat the individual types, but an approach that simultaneously treats all types present is preferred.

对于IIIbAVM,既能够经动脉途径治疗也可以经皮穿刺治疗,如果入路和栓塞安全性都没有困难,动脉入路仍是首选,因为我们对动脉造影表现更为熟悉,很容易发现AVM旁边发出的正常血管。直接穿刺的危害是无水乙醇可以经由穿刺点渗漏到邻近软组织。治疗IIIAVM禁止经由静脉途径拟行入路。对于混合类型的AVM应多种入路方法联合应用,如有一种入路方法能解决混合病变的所有病灶,这个入路方法当然要作为首选。

Evaluation of Angiographic Studies and Clinical Data

影像资料和临床数据评估

If an AVM that had been confined to one anatomic lesion at the initial workup showed two or more different types by angiography, the AVM was considered to be a mixed type of the two or more different types present. The therapeutic outcomes were assessed according to symptoms and signs and degree of devascularization at angiography. Cure was defined as complete resolution of clinical symptoms and signs, with 100% devascularization of AVMs at angiography. Partial remission was defined as complete resolution or an improvement in clinical symptoms and signs, with 50-99% AVM devascularization at angiography. No remission was defined as an improvement or no change in clinical symptoms and signs, with<50% devascularization at angiography. Aggravation was defined as a worsening of clinical symptoms and signs, regardless of the degree of AVM devascularization at angiography. Cure and partial remission were considered to be effective (successful) therapeutic outcomes of ethanol embolization.

如果一个实体病变的AVM造影发现包括两种不同的类型,就可诊断为包括两种及两种以上病变的混合类型。效果的评估通过症状、体征和造影时血管床的减少程度来判定。临床症状和体征完全消失、造影时病变血管完全消失为治愈,临床症状和体征改善甚至完全消失、血管造影50-99%病变血管消失为部分缓解,临床症状和体征无改善、血管造影病变血管减少小于50%为无缓解,症状或体征恶化,不管病变血管减少多少均为进展,其中治愈和部分缓解被认为是临床有效。

Results

结果

Results of assessing the angiographic types of the 66 AVMs based on the classifications are given in Table 1. The most common form of AVMs was type IIIb (45%, 30/66 AVMs). There were no type I AVMs. Twenty-one (32%) AVMs were categorized into the mixed form. Fifty-one (77%) AVMs had a type IIIb component, 22 (33%) AVMs had a type II component, and 14 (21%) AVMs had a type IIIa component.

表格1有基于新的分类系统的66例患者资料,最常见的类型是IIIbAVM45%, 30/66),没有IAVM21个病变(32%)被分类到混合型,51个病变(77%)包含IIIbAVM22个病变(33%)包含IIAVM14个病变包含IIIaAVM

Therapeutic Outcome

治疗效果

The therapeutic outcomes according to the types of AVMs are also summarized in Table 1. Ethanol embolization was effective in 49 (cure 21, partial remission 28) (74%) of 66 patients (Figs. 3 and 4). Of the 19 patients waiting for further treatment, 9 patients were in the partial remission group and 10 patients were in the no remission group.

With statistics, the therapeutic outcome for type II AVMs was better than for any other types (P < 0.05). No statistical difference in the therapeutic outcomes was found between the other types of AVMs (P > 0.05).

依据AVM分型总结的治疗效果如列表1,无水乙醇硬化栓塞治疗66例患者,49例有效(其中21例治愈,28例部分缓解)(74%)。19位需要再次治疗的患者,其中9属于部分缓解组,10例属于无缓解组。

统计下来,IIAVM治疗效果最好(P<0.05),其他各类型之间治疗效果无统计学差异(P>0.05)。

Complications并发症
Complications related to ethanol embolotherapy according to the angiographic types of 66 AVMs are detailed in
Table 2. Thirty-two (48%) of 66 patients had complications and there was no procedure-related mortality. Fifty-three (21%) complications occurred in 255 staged embolotherapy procedures. Four patients had major and minor complications together during the procedures. There were 43 minor complications in 28 patients (43/255 sessions, 17%; 28/66 patients, 42%). There were 10 major complications in 8 patients (10/255 sessions, 4%; 8/66 patients, 12%).

无水乙醇硬化栓塞治疗66AVM患者的并发症根据血管造影类型详细列举于表232位患者出现并发症,无操作相关的死亡病例。255个分阶段栓塞治疗,53个出现并发症。4例患者同时出现轻微并发症和严重并发症,28位患者发生43个轻微并发症(43/255次治疗,17%28/66例患者,42%),8例患者出现10个主要并发症(10/255次治疗,4%8/66例患者,12%)。

3.

Figure 3 A 21-year-old male with type II AVM in the left calf. (A-C) Pretreatment posteroanterior angiography (arterial, late arterial, venous phase) shows plexiform arteriolar component (arrows) and a large venous component (arrowheads). (D) On superselective angiography, the components of a type II AVM can be easily identified.

321岁男性患者,左侧小腿IIAVMA-C图分别为早动脉期-晚动脉期-静脉期显示网丛状的动脉成分和一个大的静脉成分,D图显示超选择动脉造影明确IIAVM

4.

Figure 4 A 42-year-old male with type IIIb AVM in the left calf. (A and B) Posteroanterior angiography (arterial and venous phase) shows multiple arteriolar and venulous components of the nidus. (C and D) Posteroanterior angiography (arterial and venous phase) after six embolotherapy sessions by the transarterial and direct puncture approaches shows a obliteration of 50% of the AVM.

4.  42岁男性左小腿IIIbAVM,正位造影(A图为动脉期,B图为静脉期)显示包含多个动脉成分和静脉成分的病灶,CD图显示经过6次经动脉和经皮穿刺治疗病灶一半已经消失。

2.躯干和肢体66AVM无水乙醇硬化栓塞治疗并发症发生情况

造影特点

病人数

治疗次数

并发症数人

并发症数

主要并发症

轻微并发症

II

13

33

4

8

异位栓塞(2

脑梗死(1)膀胱梗死(1

皮肤坏死(4

IIIa

2

3

1

2

皮肤坏死(2

IIIb

30

85

16

24

异位栓塞(1

感染1

皮肤坏死(13

水泡(8

一过性神经损伤(1

IIIIIb

9

42

3

7

永久神经损害(2

感染1

皮肤坏死(3

一过性神经损伤(1

IIIaIIIb

12

92

8

12

急性肾功能衰竭1

皮肤坏死(9

水泡(2

总计

66

255

32

53



李明省
李明省 主治医师
郑大一附院 血管外科
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