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先天性血管畸形的高级管理:多学科方法。

Advanced management of congenital vascular malformations: a multidisciplinary approach.

作者信息

Lee Byung Boong, Bergan John J

机构信息

Department of Surgery, Sungkyunkwan University School of Medicine & Samsung Medical Center, Seoul, South Korea.

出版信息

Cardiovasc Surg. 2002 Dec;10(6):523-33. doi: 10.1016/s0967-2109(02)00072-8.

DOI:10.1016/s0967-2109(02)00072-8
PMID:12453680
Abstract

INTRODUCTION

Management of congenital vascular malformations (CVM) remains a major challenge because treatment carries a substantial risk of morbidity and recurrence of the fundamental problem. The new classification of CVM allows a multidisciplinary approach to the treatment with full integration of the several treatment modalities. The multidisciplinary approach was introduced at our CVM Clinic in 1995 and this report summarizes our results to date.

PATIENTS AND METHODS

The CVM Clinic is attended by 15 different specialties. These allow application of advanced diagnosis and treatments. The interdisciplinary consultation allows proper application of the various treatment modalities including embolosclerotherapy and surgical therapy. The embolosclerosants utilized are absolute ethanol and N-butyl cyanoacrylate (NBCA). These are used for venous malformations (VM), arteriovenous shunting malformations (AVM), and hemolymphatic malformations (HLM). These agents are used independently and as preoperative adjuncts. Among the 438 patients treated between September 1995 and September 1999, there were 99 patients treated with combinations of embolosclerotherapy. There were 286 sessions, 252 of which were for ethanol sclerotherapy and 247 of these employed ethanol alone and five were combined with NBCA. Independent embolotherapy with NBCA was instituted more recently and has been used in 28 sessions. Perioperative embolosclerotherapy has been performed in 43 sessions, mostly as preliminary preparation to reduce subsequent surgical morbidity. Followup assessment of immediate and interim results after completion of multisession therapy has been done using combinations of noninvasive diagnostic testing.

RESULTS

The immediate success rate of embolosclerotherapy has been 94.7% (271/286 sessions). There has been an immediate 5.2% failure (15/286 sessions). Failures have largely been due to forced abandonment of the sclerosing procedure due to risk of deep venous thrombosis. Interim results though short-term success following completion of multisession therapy, average 3.2 sessions per patient, were satisfactory. Complications, mostly skin damage from embolosclerotherapy were experienced in 31 patients during the 286 sessions performed on 99 patients. These skin complications were discussed and accepted by the multidisciplinary team which recognized unavoidable morbidity accompanying the ethanol therapy when applied to superficially located lesions. The overall morbidity included complication rate per session (14.7%, 42/286) and 31.3% per patient (31/99). Recovery from the skin complications has been mostly spontaneous but one case in which peroneal nerve palsy occurred became permanent. There has been no recurrence of the lesions treated successfully, and this has been confirmed through a battery of noninvasive testing. The average follow-up period after completion of multisession therapy is relatively short with 10.6 months (6.0-32 months) only to meet the condition as interim results. Fourteen patients have undergone surgical ablation after preoperative embolosclerotherapy and the surgical morbidity has been minimal.

CONCLUSION

An accurate diagnosis and multidisciplinary treatment strategy for management of CVMs can improve overall treatment success with a reduced morbidity and recurrence over conventional approaches. This study reviews current trends in contemporary diagnosis and clinical management of congenital vascular malformations (CVM) of the peripheral vascular system emphasizing our new multidisciplinary approach.

摘要

引言

先天性血管畸形(CVM)的治疗仍然是一项重大挑战,因为治疗存在相当大的发病风险以及基本问题的复发风险。CVM的新分类允许采用多学科方法进行治疗,将多种治疗方式充分整合。1995年我们的CVM诊所引入了多学科方法,本报告总结了我们迄今为止的结果。

患者与方法

CVM诊所由15个不同专业的科室参与。这些科室能够应用先进的诊断和治疗方法。跨学科会诊使得包括栓塞硬化疗法和手术治疗在内的各种治疗方式得以恰当应用。所使用的栓塞硬化剂为无水乙醇和氰基丙烯酸正丁酯(NBCA)。这些药物用于静脉畸形(VM)、动静脉分流畸形(AVM)和淋巴管血管畸形(HLM)。这些药物可单独使用,也可作为术前辅助用药。在1995年9月至1999年9月期间治疗的438例患者中,有99例患者接受了栓塞硬化疗法联合治疗。共进行了286次治疗,其中252次为乙醇硬化疗法,其中247次仅使用乙醇,5次联合使用NBCA。近期开始采用独立的NBCA栓塞疗法,已用于28次治疗。围手术期栓塞硬化疗法已进行了43次,主要作为初步准备以降低后续手术的发病率。在多疗程治疗完成后,使用多种非侵入性诊断测试组合对即时和中期结果进行了随访评估。

结果

栓塞硬化疗法的即时成功率为94.7%(271/286次治疗)。即时失败率为5.2%(15/286次治疗)。失败主要是由于存在深静脉血栓形成风险而被迫放弃硬化治疗程序。中期结果虽然在多疗程治疗完成后短期成功,平均每位患者3.2次治疗,但结果令人满意。在对99例患者进行的286次治疗中,有31例患者出现并发症,主要是栓塞硬化疗法导致的皮肤损伤。多学科团队对这些皮肤并发症进行了讨论并认可,该团队认识到乙醇疗法应用于浅表病变时会伴随不可避免的发病率。总体发病率包括每次治疗的并发症发生率(14.7%,42/286)和每位患者的发生率31.3%(31/99)。皮肤并发症大多可自发恢复,但有1例发生腓总神经麻痹并遗留永久性损伤。成功治疗的病变未出现复发,这已通过一系列非侵入性测试得到证实。多疗程治疗完成后的平均随访期相对较短,仅10.6个月(6.0 - 32个月),以符合中期结果的条件。14例患者在术前栓塞硬化疗法后接受了手术切除,手术发病率极低。

结论

针对CVM的准确诊断和多学科治疗策略可提高总体治疗成功率,与传统方法相比,发病率和复发率降低。本研究回顾了周围血管系统先天性血管畸形(CVM)当代诊断和临床管理的当前趋势,强调了我们新的多学科方法。

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