1例巨大原发性下腔静脉平滑肌肉瘤切除术的手术配合

下腔静脉(inferior vena cava,IVC)平滑肌肉瘤是十分罕见的恶性肿瘤[1-7],自1871年首次报道至今,文献报道[2～7]不足300例.IVC平滑肌肉瘤对放疗、化疗不敏感,手术切除是唯一有效的方法[1～7],而肿瘤整块切除及必要的下腔静脉重建是治疗下腔静脉平滑肌肉瘤的有效方式[8].我室2010年4月配合完成1例巨大原发性下腔静脉平滑肌肉瘤切除术,现报道如下.     

布加综合征的超声诊断

目的探讨布加综合征的声像图表现及超声对布加综合征的诊断价值。方法分析31例经下腔静脉造影确诊的布加综合征病例的超声图像。结果肝静脉阻塞或闭塞（型）6例,下腔静脉阻塞或闭塞（型）22例,肝静脉和下腔静脉混合病变（型）3例。本组中超声诊断准确率93.55%。结论超声可以明确布加综合征血管病变的部位、范围及类型,准确反映下腔静脉、肝静脉的血流颜色、方向及流速曲线的变化,对诊断具有重要参考价值,可为治疗提供可靠信息。     

布-加综合征的影像诊断及综合介入治疗

目的回顾性分析31例门诊疑诊为布加综合征患者的诊疗过程,探讨布加综合征的影像学诊断方法及影响介入疗效的因素。方法31例门诊疑诊为布加综合征的患者行DSA,17例证实为布加综合征,其中16例行介入治疗。结果16例行介入治疗的病人技术上均获成功。治疗采用单纯球囊扩张7例,放置下腔静脉支架4例(5枚),放置肝静脉支架1例,下腔静脉支架结合下腔静脉滤器1例,单纯溶栓1例,行TIPSS术2例。术后30天症状明显改善13例,无变化1例,死亡2例。结论超声可作为布加综合征的筛查手段,最终确诊要依靠DSA。介入术后开通的静脉血管再闭塞是影响布加综合征疗效的主要因素。     

8例布加综合征根治术的护理

布加综合征是由于肝静脉肝段下腔静脉回流受阻引起的综合征。其临床主要表现为肝脾肿大、腹水和下肢浮肿等。我科1993年9月设计了在据低温体外循环下实施布加综合征根治术，既在无血状态直视下切除下腔静脉及肝静脉的病灶。至1995年8月已行手术8例。虽然本术式只开胸不开腹，创伤小；无血直视下手术，操作方便，手术安全；但由于布加综合征患术前病情较重，加之体外循环深低温的影响，术后血液动力学明显改变。所以术前术后的护理较其他布加征手术尤为重要。现将我们的护理经验总结如下：     

一例下腔静脉平滑肌肉瘤围手术期护理体会

下腔静脉平滑肌肉瘤（Leiomyosarcoma of the inferior vena cava）是一种极其罕见的起源于下腔静脉平滑肌细胞的恶性肿瘤［1］，但也是下腔静脉平滑肌最常见的原发性肿瘤［2］，占软组织恶性肿瘤的0．5％［3］。自1871年Pert首次报道到1996年，世界范围内仅报道218例［4］，截至2009年，世界文献报道约300例［5 ］。我院血管外科2013年7月收治1例下腔静脉平滑肌肉瘤患者，经过积极、有效的治疗和护理，患者得以成功救治。现报告如下。     

彩色多普勒超声对布加综合征诊断价值

目的:探讨彩色多普勒超声对布加综合征的诊断与应用价值.方法:回顾性分析37例布加综合征患者的彩色多普勒声像图资料.结果:37例经彩色多普勒超声检查诊断为布加综合征;10例肝静脉阻塞,27例下腔静脉阻塞.结论:彩色多普勒超声检查可作为布加综合征的首选诊断方法,能显示狭窄的部位、类型,指导临床治疗和观察手术效果.     

人工血管原位移植术治疗布加综合征的护理

布加综合征是肝静脉和（或）其开口平面以上的下腔静脉阻塞所致的伴有或不伴有下腔静脉高压为特点的肝后性门静脉高压症。针对下腔静脉局限性狭窄或闭塞所致的布加综合征,我们用完全切除局限性狭窄或闭塞段下腔静脉、人工血管原位移植术治疗１２例。因人工血管短,短期内可完全内皮化,局部血流量大,血流笔直,形成血栓的机会少,使患者得以根治。认真、细致的护理对确保手术成功起着重要作用。现将护理体会报告如下。１　临床资料本组１２例,男９例,女３例,２２～５４岁。术前经彩超、腔静脉造影明确诊断。手术方法为将狭窄的下腔静脉…     

布加综合征直视根治术18例

目的：总结布加综合征根治术的体会。方法：在常温阻断下行下腔静脉直视根治术１８例，其中膜切除１２例，下腔静脉扩大成形６例。结果：本组患者均治愈出院。随访４～６０个月，典型表现均消失。彩色Ｂ超证实肝静脉和下腔静脉血流均通畅。结论：布加综合征直视根治术是较理想的治疗方法     

“十字三步法”手术策略治疗跨肾静脉开口的下腔静脉平滑肌肉瘤的可行性

目的：探讨采用“十字三步法”策略手术治疗跨肾静脉开口下腔静脉平滑肌肉瘤的疗效及安全性。方法：回顾性分析2020年10月至2022年8月期间，采用“十字三步法”策略手术治疗跨肾静脉开口下腔静脉平滑肌肉瘤病例9例，收集患者一般情况、影像资料、手术记录及术后并发症情况，比较术前、术后肾功能指标，采用门诊或电话方式进行随访。结果：9例患者均完成手术顺利出院，其中单侧肾脏切除4例，肾静脉人工血管重建4例，下腔静脉人工血管重建1例，手术平均出血量1966ml，平均手术时间179min，总体及各亚组患者术前与术后2周血肌酐水平无显著性差异。结论：“十字三步法”手术策略将复杂多变的下腔静脉肿瘤手术步骤标准化，具有可复制性，有助于缩短术者学习曲线，手术总体安全可靠，术后患者肾功能恢复良好。     

布加综合征直视根治术18例

目的，总结布加综合征根治术的体会，方法：在常温阻断下行下腔静脉直视根治术１８例，其中膜切除１２例，下腔静脉扩大成形６例。结果：本组患者均治愈出院。随访４－６０个月，典型表现均消失。彩色Ｂ超证实肝静脉和下腔静脉血流均通畅。结论：布加综合征直视根治术是较理想的治疗方法。     

Obstruction of the inferior vena cava in the hepatic portion and hepatocellular carcinoma

High incidence of hepatocellular carcinoma in patients with obstruction of the inferior vena cava in the hepatic portion (Budd-Chiari syndrome) was previously pointed out by us from the review of Japanese literature of autopsied cases. This was confirmed by the follow-up study of 16 patients with obstruction of the inferior vena cava in the hepatic portion hospitalized in 1958 to 1974. Follow-up information was available on 13 of the 16 patients. Deaths due to hepatocellular carcinoma occurred in 6 patients (46%), and those due to other causes in 4 patients (31%). Three patients were alive. Hepatocellular carcinoma occurred most frequently in the patients who were found to have obstruction of the inferior vena cava at less than 44 years of age and were followed up for more than 10 years.     

布-加综合征合并上腔静脉阻塞诊治分析

目的探讨布一加综合征合并上腔静脉阻塞的诊断与治疗方法。方法本组3例布一加综合征术前均经腹部彩超及磁共振静脉成像检查确诊，上腔静脉阻塞经上腔静脉造影证实。3例下腔静脉均狭窄闭塞行球囊扩张成形术；例1上腔静脉入右房口处狭窄采用球囊单纯扩张，例2、3因无明显上腔静脉阻塞症状且上腔静脉完全闭塞导丝无法通过而未行介入处理。结果3例介入手术后下腔静脉压力分别由术前的23．33、25．88和17．55mmHg降至9．60、9．60和7．20mmHg。例1上腔静脉压力由术前16．58mmHg降至术后6．98mmHg。3例术后皆恢复顺利，出院。随访症状完全消失、肝肾功能恢复正常。结论对布一加综合征患者术前应充分了解上腔静脉通畅情况，避免漏诊上腔静脉阻塞。对上腔静脉阻塞症状较轻或无症状者可不予处理，症状较重者应根据病因进行治疗。     

Numerical simulation of hemodynamics in membranous obstruction of the suprahepatic inferior vena cava based on a subject-specific Budd-Chiari syndrome model

BackgroundThis study was performed to determine the hemodynamic changes of Budd-Chiari syndrome when the inferior vena vein membrane is developing.MethodsA patient-specific Budd-Chiari syndrome vascular model was reconstructed based on magnetic resonance images using Mimics software and different degrees (16%, 37%, and 54%) of idealized membrane were built based on the Budd-Chiari syndrome vascular model using Geomagic software. Three membrane obstruction Budd-Chiari syndrome vascular models were established successfully and fluent software was used to simulate hemodynamic parameters, including blood velocity and wall shear stress.FindingsThe simulation results showed that there is low velocity and a low wall shear stress region at the junction of the inferior vena cava and the branches of the hepatic veins, and swirl may occur in this area. As the membrane develops, the size of the low velocity and low wall shear stress regions enlarged and the wall shear stress was increased at the membrane region. There was a significant difference in the mean values of wall shear stress between the different obstruction membrane models (P < 0.05).InterpretationHemodynamic parameters play an important role in vascular disease and there may be a correlation between inferior vena cava wall shear force changes and the slow development process of the inferior vena cava membrane.     

Review of Budd-Chiari Syndrome

This study aims to report the Budd-Chiari syndrome clinical research status and progress that has occurred in over nearly 30 years in China, and emphasize the value of imaging in facilitating the diagnosis of Budd-Chiari syndrome based on more than 2500 cases. Findings on ultrasonography, computed tomography, magnetic resonance imaging, and digital subtraction angiography images are used to propose new Budd-Chiari syndrome types and subtypes. The new subtype classification presented here has important value for guiding interventional treatment. This study also proposes a new concept of anatomical and functional obstruction of hepatic vein that stresses the compensatory value of accessory hepatic vein and azygos vein and describes the risk of manipulation of the communication branch of inferior vena cava obstruction in interventional therapy.     

Relief of Budd-Chiari syndrome by primary placement of a metallic stent in the inferior vena cava: Case report

Summary. A case of Budd-Chiari syndrome with associated lower limb oedema due to concomitant inferior vena caval and hepatic venous thrombosis is presented. Percutaneous placement of a Wallstent through the occluded vena cava resulted in resolution of both the lower limb oedema and the hepatic vein thrombosis. In this instance recannalization of the inferior vena cava alone resulted in improvement of his liver function.     

Computational fluid dynamics simulation of time-resolved blood flow in Budd-Chiari syndrome with inferior vena cava stenosis and its implication for postoperative efficacy assessment

BackgroundThis study aimed to adopt computational fluid dynamics to simulate the blood flow dynamics in inferior vena cava stenosis based on time-dependent patient-specific models of Budd-Chiari syndrome as well as a normal model. It could offer valuable references for a retrospective insight into the underlying mechanisms of Budd-Chiari syndrome pathogenesis as well as more accurate evaluation of postoperative efficacy.MethodsThree-dimensional inferior vena cava models of Budd-Chiari syndrome patient-specific (preoperative and postoperative) and normal morphology model were reconstructed as per magnetic resonance images using Simpleware. Moreover, computational fluid dynamics of time-resolved inferior vena cava blood flow were simulated using actual patient-specific measurements to reflect time-dependent flow rates.FindingsThe assessment of the preoperative model revealed the dramatic variations of hemodynamic parameters of the stenotic inferior vena cava. Moreover, the comparison of the preoperative and postoperative models with the normal model as benchmark showed that postoperative hemodynamic parameters were markedly ameliorated via stenting, with the attenuation of overall velocity and wall shear stress, and the increase of pressure. However, the comparative analysis of the patient-specific simulations revealed that some postoperative hemodynamic profiles still bore some resemblance to the preoperative ones, indicating potential risks of restenosis.InterpretationComputational fluid dynamics simulation of time-resolved blood flow could reveal the tight correlation between the hemodynamic characteristics and the pathological mechanisms of inferior vena cava stenosis. Furthermore, such time-resolved hemodynamic profiles could provide a quantitative approach to diagnosis, operative regimen and postoperative evaluation of Budd-Chiari syndrome with inferior vena cava stenosis.     

Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome

Severe hepatic venous outflow obstruction and its manifestations often are recorded under the label "Budd-Chiari syndrome." Unfortunately, this label is ambiguous; it does not clearly identify the site of the lesion (hepatic veins versus inferior vena cava), its morphologic features (thrombotic versus nonthrombotic), or its cause. In the literature, implied or expressed definitions vary. Use of a standardized topographic and pathogenetic classification of hepatic venous outflow obstruction would enable investigators to group patients with comparable conditions, as required for therapeutic trials, prognostic evaluations, and studies of pathogenetic pathways. Review of our own cases revealed that hepatic venous outflow obstruction involving large hepatic veins is usually thrombotic and that isolated obstruction of the inferior vena cava or of small hepatic veins is usually nonthrombotic. Application of such a classification seems feasible and may yield useful results.     

超声引导下下腔静脉内支架成形术

超声导向下，行下腔静脉球囊扩张并内支架置入术，治疗布－加氏综合症６例，全部成功，支架置入后，狭窄部直径达为１．５－１．８ｃｍ，下腔静脉压力由４１ｃｍＨ２Ｏ降至２０．５ｃｍＨ２Ｏ，所有患者的症状体征者消失或明显改善。术中未出现明显并发症。本技术避免了Ｘ线下操作的放射照射及造影剂注入，设备简单、费用氏，治疗布－加氏综合症切实可行。     

Fatal tumor thrombosis due to an inferior vena cava leiomyosarcoma in a patient with antiphospholipid antibody syndrome

We describe a patient with antiphospholipid antibody syndrome (APS) who died because of relentless inferior vena cava (IVC) tumor thrombosis due to an unsuspected leiomyosarcoma. Laboratory confirmation for APS was provided by functional identification of a lupus anticoagulant and anticardiolipin IgG and anti-beta2-glycoprotein I IgM antibodies. Although sensitive for detecting vascular obstruction, radiocontrast venography and magnetic resonance imaging and angiography detected the IVC thrombosis but failed to distinguish its malignant nature. Concomitant refractory thrombocytopenia prevented further invasive diagnostic and therapeutic maneuvers for progressive, severe IVC thrombosis unresponsive to aggressive treatment of APS. Deep venous thrombosis refractory to anticoagulant and immunomodulatory therapies in a patient with APS may be due to a concomitant underlying malignancy, such as a leiomyosarcoma, causing vascular obstruction.