Treatment of venous malformations （VM）： The cutting edge knowledge

Venous malformations (VMs) are problematic common vascular malformations that are challenging even for experienced physicians. Several treatments are available including sclerotherapy, surgery, laser or combinations of these procedures. As none of these is specific, we still need therapies that would allow to cure these patients without complications. Hopefully, the unraveling of the causative defects of VMs will give us new tools for the management of this difficult condition.     

Vascular malformations of the orbit (lymphatic,venous, arteriovenous): Diagnosis,management and results

Vascular malformations are often found inside the orbit. Isolated venous malformations (frequently misnamed as cavernous hemangiomas) are the most frequent among these. However, also lymphatic and arteriovenous malformations can affect the orbit. The complex anatomy of the orbit and the fact that its content easily suffers from compartmental syndrome explain why treating orbital vascular malformations can be challenging and technically demanding.In this study, two institutions have retrospectively collected their cases, consisting in a total of 69 vascular malformations of the orbit. Each type of malformation has been evaluated separately in terms of diagnosis, indications for treatment, techniques and outcomes. Moreover, the authors have analyzed in detail venous malformations, identifying three different types, named orbital venous malformation (OVM) 1, 2 and 3. These behave differently from each other, and a prompt differential diagnosis is mandatory to pose correct indications, minimize risks and improve results.Overall, surgery was the technique of choice for OVM1, microcystic lymphatic malformations (LM) and arteriovenous malformations (AVM). A pure transnasal approach with mass removal and reconstruction of the medial wall with polyethylene sheets was chosen for OVM1 (intra- or extraconal) located in the medial or superomedial compartment.Sclerotherapy had a role in treating macrocystic LM and OVM3.     

Intraosseous venous malformation of the craniofacial region: diagnosis and management

Vascular lesions mainly affect soft tissues, and less than 1% affect bone. In 1982, they were categorised by Mulliken and Glowacki as haemangiomas or vascular malformations, and an updated classification was subsequently published by the International Society for the Study of Vascular Anomalies. These lesions, however, continue to be termed haemangiomas and there is little attempt to differentiate between them. We report eight cases of intraosseous venous malformation that were inappropriately labelled as haemangioma by clinicians, pathologists, and radiologists. We highlight tailored management, and describe the clinical features, results of investigations to aid accurate designation (histological and immunohistochemical, including GLUT1 staining and cross-sectional imaging), and outcomes.     

Current concepts in diagnosis and treatment of venous malformations

IntroductionVenous malformations are the most common type of vascular malformation, usually detected at birth or during puberty. By occurring during human growth or through localized trauma, pain, functional impairment and aesthetic disfigurement is often observed.Ultrasonography, Doppler flow Imaging, and Magnetic Resonance Imaging are the most informative techniques which reveal the extent of tissue involvement and differentiate between high and low flow anomalies. Therapeutic options for treatment of venous malformations are sclerotherapy with alcohol, ethoxysclerol and bleomycin, laser therapy (Nd:YAG), surgery and combined therapeutic modalities. The aim of percutaneous sclerotherapy is the successive reduction of the volume of the lesion by aseptic inflammation.Patients and methodsThis is a review of 51 patients with venous malformation treated by the Interdisciplinary Center for Vascular Anomalies at the University Hospital Tübingen, (Germany), from July, 2002 until January, 2007. The mean age of first consultation in our outpatient department was 26.4 years (median).12 patients were treated by sclerotherapy with highly concentrated alcohol, 9 by surgery, and 7 by laser therapy. In some cases we combined different treatments. 9 patients had only sclerotherapy, while 3 had a combination of pre-operative sclerotherapy and surgery.ResultsWe obtained positive results in patients treated with sclerotherapy and combined sclerotherapy and surgery.ConclusionSclerotherapy is safe (under fluoroscopic control), efficient, and can be repeated multiple times. Therefore, it should be considered as first-line treatment in venous malformations. A combination of a sclerotherapy with surgery is also useful in many cases.     

生物胶联合OK-432和平阳霉素治疗面颈部巨大静脉畸形

目的：评价生物胶联合OK-432和平阳霉素治疗面颈部巨大静脉畸形的临床疗效。方法：26例面颈部巨大静脉畸形患者,男11例,女15例;年龄4～23岁,平均年龄11.8岁。通过注射生物胶联合OK-432和平阳霉素进行治疗。所有病例均只接受1次治疗。结果：除术区2～3周肿胀外,均无严重并发症出现。随诊6～12个月（平均8.4个月）,19例治愈,5例显效,2例有效,无无效病例。结论：生物胶联合OK-432和平阳霉素经皮硬化术治疗面颈部巨大静脉畸形,效果良好,并且简单、安全和可靠。     

Diagnosis and management of venous malformations: An overview

Venous malformations pose some of the most difficult challenges in the practice of medicine today. Clinical manifestations of these lesions are extremely protean. Because of the rarity of these lesions, most clinicians have limited experience in their diagnosis and management, which augments the enormity of the problem and can lead to misdiagnoses, inadequate treatment, high complication rates, and poor patient outcomes. Vascular malformations are best treated in medical centers where patients with these maladies are seen regularly and the team approach is used. The occasional embolizer will never gain enough experience to treat these problematic lesions adequately. More importantly, when complications do occur, the morbidity of that complication is worsened because of this lack of experience and the absence of an experienced team of physicians. All too frequently, the patient ultimately pays for a physician＇ s initial enthusiasm, inexperience, folly, and lack of necessary clinician backup. A cavalier approach to the management of venous malformations will always lead to significant complications and dismal patient outcomes. These patients should be referred to centers that regularly treat vascular malformations, appropriately manage complications in a timely manner, and routinely deal with the dilemmas they present. Only in this fashion can significant experience be gained, improved judgment in managing these lesions develop, and definitive appropriate statements in the treatment of vascular anomalies evolve.     

腮腺区静脉畸形翻瓣激光术的操作探讨

目的:探讨翻瓣Nd:YAG激光术处理腮腺区静脉畸形时的外科操作难点及注意事项。方法:回顾总结近3a来临床诊治的84例患者,全部确诊为腮腺区静脉畸形。均采用全麻下翻瓣激光治疗,手术前后CT、MRI等影像学检查。术后作面神经评判,术中统计出血量。结果:84例患者中,有5例为暂时性面瘫,2个月后自主恢复。1例为永久性面瘫,占1.19%,平均失血约为200ml。4例接受术中、术后输血。结论:腮腺区静脉畸形翻瓣激光术中,只要注意精细解剖操作,合理运用激光照射方式,可获得保存面神经,减少出血的理想效果。     

口腔颌面部静脉畸形诊治：经验与进展

静脉畸形是临床上最常见的先天性血管畸形,可发生于全身各个部位。其病变位置及范围的多样性,使得静脉畸形的诊断和治疗富有挑战性。目前,口腔颌面部静脉畸形的治疗方法主要包括硬化治疗、手术治疗、激光治疗等。本文通过对近几年文献的回顾,结合作者经验,对静脉畸形诊断和治疗的相关进展进行了总结。     

高频电凝治疗舌脉管畸形34例临床观察

目的：总结高频电凝治疗舌脉管畸形的疗效。方法：采用高频电凝治疗舌脉管畸形患者34例,根据病变部位、大小、深度,调整输出能量控制,一般在10～50W。结果：治疗后经6个月～3a随访,舌静脉畸形治愈率68.75%,显效率25.00%;舌淋巴管畸形治愈率66.67%,显效率22.22%。结论：高频电凝是治疗舌脉管畸形的一种微创、安全、有效的新方法。     

Intraoral venous malformation with phleboliths

The most common type of vascular malformation is the venous malformation and these are occasionally associated with phleboliths. We report a case of a 45 year old woman with intraoral venous malformation with phleboliths.     

射频消融术用于治疗面颈部脉管畸形

目的:观察射频消融技术治疗面颈部脉管畸形的效果.方法:回顾2009- 07～2010- 10间32 例患者的临床资料.32 例患者中16 例为静脉畸形,10 例为动静脉畸形,6 例为淋巴管畸形.患者接受单纯射频消融治疗或射频消融辅助治疗.通过临床观察、B超、磁共振、三维CT血管造影等评价治疗效果.随访时间3～12 个月...     

两种硬化治疗方法对面颈部巨大静脉畸形的疗效比较

目的比较生物胶联合OK-432和平阳霉素与区室化联合OK-432和平阳霉素两种硬化治疗方法对面颈部巨大静脉畸形的临床疗效。方法选择63例面颈部巨大静脉畸形患者,随机分为A、B两组,A组30例,B组33例。A组以生物胶联合OK-432和平阳霉素经皮共同注射;B组对病灶行区室化后,经皮注射OK-432和平阳霉素。两组均经气管插管全麻下进行,相隔1个月注射1次,连续注射1～5次。比较2组的疗效和并发症。结果随诊6～12个月,平均8.2个月。A组平均治疗时间1.5个月,平均治疗次数2.5次,20例治愈,6例显效,2例有效,2例部分有效,没有无效病例;治愈率为66.67%,总有效率为100%。B组平均治疗时间3.5个月,平均治疗次数4.3次,13例治愈,9例显效,9例有效,2例部分有效,没有无效病例,治愈率为33.33%,总有效率为100%。A、B两组治愈率差异有统计学意义(χ2=21.01,P<0.01);A、B两组患者均有局部肿胀,无严重并发症出现,差异无统计学意义(χ2=1.56,P>0.05)。结论生物胶联合OK-432和平阳霉素经皮硬化术治疗面颈部巨大静脉畸形疗程短、疗效良好、安全、可靠。     

平阳霉素和泼尼松龙及鱼肝油酸钠联合注射治疗颌面部静脉畸形60例报道

目的：评价平阳霉素和泼尼松龙及鱼肝油酸钠联合注射治疗颌面部静脉畸形的疗效。方法：采用平阳霉素和泼尼松龙及鱼肝油酸钠联合病变内注射治疗60例颌面部静脉畸形患者。1周注射1次，3-5次为1个疗程，治疗结束后对疗效进行评价。结果：60例患者平均疗程3周，治疗完成后随访0．5～2a，治愈和显效率达94％，有效率为100％。结论：平阳霉素和泼尼松龙及鱼肝油酸钠联合注射治疗颌面部静脉畸形安全、廉价、高效、简便，疗程适中。     

静脉畸形患者围术期D-二聚体、纤维蛋白原的动态变化及临床意义

目的 动态监测、比较围术期静脉畸形和非静脉畸形患者的D-二聚体、纤维蛋白原水平变化,分析探讨其在不同部位、面积、深度和手术条件下的差异,为指导静脉畸形的临床诊治提供依据。方法：2011年10月—2013年3月,对82例患者进行围术期纤维蛋白原、D-二聚体水平动态监测,分别于术前,术后第2、第5和第7 天进行。非静脉畸形病例30例（A组）,静脉畸形患者52例（B组）。B组根据受累部位不同分为L1组（上肢患者,14例）、L2组（下肢患者,18例）、L3组（头颈部患者,8例）、L4（躯干患者,7例）和L5组（累及2个以上解剖部位,5例）。根据肌肉是否受累,分为M1组（肌肉受累,36例）, M2组（肌肉不受累,16例）。根据手术方式分为S1组（部分切除,44例）和S2组（全切除,8例）。记录B组所有患者病变面积,以%体表面积计算。应用SPSS18.0软件包对分组定量资料进行t检验或方差分析,两定量资料间作线性相关分析。结果：A组和B组患者术前、术后第2、第5、第7 天的D-二聚体和纤维蛋白原水平比较均有显著差异（P均<0.05）。M1、M2 2组间术前、术后第2、第5和第7天的D-二聚体水平显著改变（P均<0.05）;术后第7天的纤维蛋白原水平有显著差异（P<0.05）。L1~5组间术前、术后第2天 、第7天的D-二聚体水平变化有统计学差异（P均<0.05）。S1、S2 2组间术后第2、术后第5和术后第7天的D-二聚体变化水平比较有显著差异（P<0.05）,2组间术后第5天和术后第7天的纤维蛋白原变化差异显著（P均<0.05）。定量相关分析提示,D-二聚体水平和纤维蛋白原水平呈负相关,病变面积和围术期D-二聚体水平呈正相关,与围术期纤维蛋白原水平呈负相关。结论：D-二聚体水平升高可作为静脉畸形患者的辅助性诊断指标;多个解剖部位、深部肌肉广泛受累的静脉畸形患者,尤其是臀部、下肢大面积弥漫性病变是围术期发生D-二聚体水平异常升高的危险因素,该类患者术后常继发纤维蛋白原明显降低,导致严重的凝血功能紊乱,手术治疗须慎重,以免造成严重后果。     

Comprehensive treatment of massive macroglossia due to venous and lymphatic malformations

This study evaluated the outcomes of comprehensive treatment for massive macroglossia. Eleven patients with massive macroglossia due to venous malformations (VMs) and lymphatic malformations (LMs) underwent incision excision of two-thirds of the central wedge of the tongue; two of these patients underwent resection of lesions in the lower lip or floor of the mouth during initial treatment. In subsequent treatment, three patients underwent orthognathic surgery or resection of lesions in the lower lip and submental region, and four patients underwent one or two sclerotherapies. No patient had complications resulting from anaesthesia or surgery. Patients were followed up at 6 months postoperative, and all signs associated with the lesions had disappeared. The mean follow-up duration was 30.8 months. The aesthetic outcome was excellent for nine patients and satisfactory for two. Nine patients could take a soft diet and two a solid diet. Eight patients presented normal speech and three presented intelligible speech. Comprehensive treatment including incision excision of two-thirds of the central wedge of the tongue, as well as orthognathic surgery, the resection of lesions in the oral and maxillofacial region, and sclerotherapy, is effective for patients with massive macroglossia due to VMs and LMs.     

聚桂醇联合平阳霉素治疗120例静脉畸形疗效观察

目的:通过与平阳霉素局部注射治疗静脉畸形对比,观察聚桂醇联合平阳霉素局部注射治疗静脉畸形的临床效果.方法:选取口腔颌面-颈部静脉畸形患者120例,病变直径4.0～6.0 cm,随机分为实验组(60例)和对照组(60例),实验组采用聚桂醇联合平阳霉素局部注射治疗,两者间隔2周交替使用;对照组仅使用平阳霉素进行局部注射治疗,间隔2周注射1次,观察治疗后病变缩小程度、局部颜色、质地、体位试验变化及不良反应.采用SPSS 11.5软件包对数据进行统计学分析.结果:根据国际疗效评价标准,第2、3、4、5次治疗后,实验组的治疗效果显著优于对照组(P＜0.05);第1、6次治疗后实验组与对照组的治疗效果未见显著差异(P＞0.05);实验组治疗有效的平均次数显著少于对照组(P＜0.05).结论:聚桂醇联合平阳霉素治疗静脉畸形与单纯使用平阳霉素相比,可提高疗效、缩短疗程,无明显不良反应.     

面颈部静脉畸形的综合治疗

目的:探讨多种方法综合治疗口腔及面颈部大面积静脉畸形的临床效果。方法:根据患者的年龄、病变部位、病变大小制订个体化治疗方案,采用多种药物联合注射,结合病变周围环形缝扎、激光、手术等方法综合治疗口腔颌面部大面积静脉畸形134例。结果:134例中治疗效果优79例(58.96%),良45例(33.58%),显效10例(7.46%),差0例。结论:采用多种方法综合治疗口腔颌面部大面积静脉畸形,对提高疗效、改善面部形态、维持局部功能、减少不良反应具有重要意义。     

副腮腺区孤立性静脉畸形10例分析

目的:探讨副腮腺区孤立性静脉畸形的临床表现、治疗与预后。方法:收集2002年至2009年收治的10例副腮腺区孤立性静脉畸形患者数据,包括人口学资料、临床表现、影像学表现、组织学和免疫组化结果、外科治疗方法和预后。结果:10例患者中,男女各5例,年龄25~70岁,平均42.8岁。病程从0.5个月~168个月不等,平均64.9个月。副腮腺区肿块大小从1.0 cm×0.8 cm~2.5 cm×2.5 cm,平均1.7 cm×1.5 cm。患者接受的影像学检查主要为超声多普勒和CT扫描。所有患者均接受不同入路下的手术切除。术后病理诊断均为静脉畸形。术后随访期间(2~94个月不等,平均41.2个月),未见复发病例。结论:对于副腮腺区孤立性静脉畸形,超声多普勒、CT扫描和细针穿刺细胞学检查,可帮助临床诊断,手术切除效果良好,复发少见。     

面颈深部静脉畸形和淋巴管畸形的彩色多普勒超声鉴别诊断

目的 分析面颈深部静脉畸形和淋巴管畸形的彩色多普勒超声特点,总结其鉴别诊断要点,探讨彩色多普勒超声在2种深部病变鉴别诊断中的意义。方法 回顾分析2012年12月—2013年12月临沂市肿瘤医院收治的经手术病理或DSA下经皮穿刺瘤体造影确诊的32例静脉畸形和29例面颈部淋巴管畸形的临床和彩色多普勒超声资料,分析静脉畸形和淋巴管畸形的彩色多普勒超声特点,以病理诊断或DSA诊断为金标准,应用SPSS 18.0软件包对数据进行统计学分析,计量特异度和敏感度。结果 32例静脉畸形病例,二维超声均显示无回声,无间隔,14例病变内见低回声血栓,11例内见高回声静脉石。多普勒超声均见周边动脉血流, PSV为(13.98±7.93) cm/s,RI为(0.71±0.83) cm/s, 30例探头加压可见静脉频谱的血流信号;29例淋巴管畸形病例,单囊者8例,多囊者10例,网格状11例,二维超声也均呈无回声,但21例病变内可见多发强回声间隔;多普勒超声2例探头加压见血流信号,PI下为静脉频谱,间隔内均可见动脉血流信号,PSV为(14.32±6.57) cm/s,RI为(0.68±0.79) cm/s。经t检验,2组PSV和RI比较无显著差异（P均>0.05）。经χ²检验2组低回声血栓、高回声静脉石、探头加压见血流信号和多发强回声间隔出现率,有显著差异（P<0.05）。探头加压见血流信号诊断静脉畸形的敏感度为93.75%,低回声血栓、高回声静脉石和探头加压见血流信号诊断静脉畸形的特异度较高,分别为96.55%、93.33%和100%。多发强回声间隔诊断淋巴管畸形的敏感度和特异度较高,分别为72.41%和100%。结论 彩色多普勒超声鉴别面颈深部静脉畸形和淋巴管畸形具有无创、价廉、准确的特点。病变内出现低回声血栓或高回声静脉石,探头加压出现血流信号支持静脉畸形诊断;病变内有多发强回声间隔,同时间隔内见动脉血流信号时支持淋巴管畸形诊断。     

西罗莫司治疗静脉畸形研究现状

静脉畸形是临床上最常见的血管畸形,大多数是由于编码TIE2或PI3K基因中的功能获得性体细胞突变引起的。复杂或顽固性静脉畸形的治疗,仍然是临床上面临的难题和挑战。西罗莫司为mTOR抑制剂,是第一个用于静脉畸形的靶向药物,其疗效和安全性近年来颇受关注。本文就西罗莫司用于治疗静脉畸形的相关问题进行综述。