多支架压栓加肠腔分流术治疗重症布加综合征的护理

目的 探讨血栓性膜型阻塞合并肝静脉堵塞型布加氏综合征的介入加手术治疗的护理方法. 方法 自1997年4月至 2000年8月,对29 例血栓性膜型阻塞性布加氏综合征患者实施多支架压栓并球囊扩张成形支架置入术,对其中11例肝静脉堵塞患者再次行肠腔分流术,回顾性研究其护理方法.结果 经过多项术前、术后护理措施的应用,无"3腔2囊管"相关并发症,严重出血性并发症,肺栓塞等严重并发症发生.随访3个月～5.5年,效果良好. 结论 对血栓性膜型阻塞性合并肝静脉堵塞型布加氏综合征,可施行多支架压栓加肠腔分流术治疗,适当的护理干预是保证手术成功的重要措施.     

多支架压栓加肠腔分流术治疗重症布加综合征

目的 :探讨血栓性膜型阻塞合并肝静脉堵塞型布加氏综合征的介入加手术治疗方法。方法 :自 1997年 4月～ 2 0 0 0年 8月 ,对 2 9例血栓性膜型阻塞性布加氏综合征患者实施多支架压栓并球囊扩张成形支架置入术 ,对其中 11例肝静脉堵塞患者再次行肠腔分流术。结果 :下腔静脉压力由术前的平均 2 7.33± 4 .12cmH2 O下降至术后的平均 11.4 2± 2 .11cmH2 O ,上腔静脉压力由术前的平均 4 .0 8± 5 1cmH2 O上升至 8.83± 2 .6 2cmH2 O ,手术后门静脉压力平均下降 2 3.12cmH2 O。无肺栓塞等严重并发症发生。随访 3个月～ 5 .5年 ,效果良好。结论 :对血栓性膜型阻塞性合并肝静脉堵塞型布加氏综合征 ,可施行多支架压栓加肠腔分流术治疗。     

肝静脉型布加综合征的介入治疗

目的评价肝静脉型布加综合征的介入治疗方法。方法30例患者经血管造影证实为肝静脉阻塞性布加综合征,对部分患者行介入治疗,并对介入治疗后的患者进行随访。结果30例患者分为中央性肝静脉阻塞（COHV,n=24）和广泛性肝静脉阻塞（EOHV,n=6）两型。24例COHV的患者接受了PTA,术后症状明显改善或消失,6例EOHV的患者选择了手术治疗。结论中央性肝静脉阻塞的布加综合征适用于介入治疗,疗效满意。     

肝静脉再堵塞型布加综合征的治疗及预防(附6例报告)

布加综合征 (BCS)治疗困难 ,以往多采用手术治疗 ,手术难度大 ,风险大。我院自 1988年 8月始开展BCS综合治疗的研究 ,共治疗各型布加综合征 6 2 2例 ,其中行介入治疗 2 6 7例。 1996年 8月— 2 0 0 2年 12月 ,对 6例肝静脉再堵塞病例进行肠腔分流术后得到治愈 ,现报告如下。1　临床资料1.1.　一般资料　本组男 4例 ,女 2例 ,年龄 2 9～ 5 1岁 ,平均37.4岁。其中 3例已行下腔静脉破膜扩张并内支架置入术 ,3例行多支架压栓治疗并内支架置入术。于术后 2～ 5年分别出现不同程度的呕血、黑便、腹腔积液、肝脾肿大和下肢指凹性水肿与足靴区色…     

布加综合征2例临床报道

布加综合征(Budd-Chiari Syndrome)系由肝静脉和(或)下腔静脉肝段狭窄或阻塞引起的以门静脉高压和(或)下腔静脉高压为临床表现的一种综合征。临床发病率低,为0.1%~1.0%,虽然此前已有多方相关报道,对本病的认识有所提高,但其临床表现复杂多变,极易误诊为肝硬化,本文对我院确诊的2例布加综合征进行临床分析,以提高基层医院业务人员对本病的认识,减少误诊,提高诊治水平。     

介入治疗布加综合征48例分析

目的回顾总结介入方法治疗布加综合征48例的临床经验,探讨布加综合征血管内介入治疗的价值。方法分析48例在造影明确诊断后行血管内介入治疗方法①下腔静脉(IVC)狭窄扩张或破膜扩张;②IVC扩张并内支架置入术;③经右颈静脉肝静脉(HV)再通成形术。结果17例共23次行IVC破膜扩张或狭窄扩张,29例次应用IVC破膜扩张或狭窄扩张后置入内支架。其中1例因弥漫性HV发育不良而转外科手术,1例术后死于急性心功能衰竭。10例HV阻塞患者中8例成功施行经右颈静脉HV再通成形术。术后IVC压力下降(1.53±0.41)kPa。结论介入治疗布加综合征安全有效,应成为治疗本病的首选方法。     

肝段下腔静脉全程显露的布加综合征根治术

目的总结肝段下腔静脉全程显露布加综合征根治术的治疗经验。方法回顾性分析了2001年9月~2006年10月在我科行肝段下腔静脉全程显露布加综合征根治术的60例患者的临床资料。结果所有患者均成功地在直视下完全切除病变,其中3例在体外循环辅助下完成,52例采用右房插管法,4例采用细胞回收机,1例采用自家回收血完成,回收血为300~4000ml。14例需输血400~2000ml,其余病例未予输血。1例于围手术期死于肾功能衰竭;1例下腔静脉血栓术后症状复发为新生隔膜,再次根治成功;1例术后下腔静脉再狭窄,无症状未予处理;其余病例术后症状体征消退。结论肝段下腔静脉全程显露布加综合征根治术视野更清晰,能更彻底地切除病变。     

布-加综合征的介入治疗

1845年，Budd最早描述了肝静脉血栓形成而造成的肝肿大、腹水等临床表现；1899年，Chiari详细描述了小肝静脉闭塞所导致的类似临床症状，称之为闭塞性静脉内膜炎。后来人们将上述发现结合在一起，形成所谓的Budd．Chiari Syndrome（BCS，布-加综合征）概念，即肝静脉血栓形成，进而出现肝静脉流出道受阻所产生的一系列临床表现。经过百余年来的认识和发展，BCS的涵义已明显扩展。目前多采用Ludwig提出的BCS概念，即发生在肝脏与右心房之间的肝静脉和（或）下腔静脉阻塞及其所产生的相应临床表现。     

体外循环下布加综合征根治术21例疗效

郑州大学第一附属医院肝胆外科采用体外循环下根治术对21例下腔静脉人右心房处长节段狭窄或隔膜下血栓形成伴肝静脉不通畅的布加综合征患者进行治疗,取得满意疗效,报道如下.     

Radical correction of Budd-Chiari syndrome

Background Interventional therapy is widely accepted as the first choice for the treatment of the Budd-Chiari syndrome but the use of radical correctional therapy should not be discarded. This study describes radical correction by controlling bleeding from distal end of pathological segment of the inferior vena cava （IVC） and discusses potential surgical errors and postoperative complications. Methods Of the 216 patients in the study, 78 were treated with simple membranectomy, 64 with dissection of the pathological segment of the IVC and vascular prosthesis or pericardial patch plasty, 60 with resection of the pathological segment of the IVC and orthotopic graft transplantation with vascular prosthesis, and 14 with resection of the occlusive main hepatic vein and its upper IVC, hepatic venous outflow plasty and vascular prosthesis orthotopic graft transplantation from the hepatic venous entrance to the IVC of right atrial ostium. Results Except 14 cases who were discharged after hepatic vein outflow plasty, four cases died postoperatively, and 198 patients were discharged without complications. The symptoms of 15 patients were relieved partially and 2 without any change. There were no deaths intraoperatively. Of the 112 cases who were followed up for 72 months, 13 suffered from a relapse. Conclusions Radical correction is a beneficial therapy in the treatment of Budd-Chiari svndrome.     

布-加氏综合征的临床影像分析(附81例报告)

对81例布-加氏综合征(BCS)患者的临床及影像表现进行了综合分析,并提出了分型标准。经DSA、彩超、CT、经皮肝穿刺活检确诊的布-加氏综合征81例。BCS患者主要临床表现为下腔静脉梗阻和门脉高压症状。胆囊壁增厚是其影像诊断的一个间接征象。     

布加综合征诊治现状

布加综合征（BCS）的病因目前尚不清楚,部分隔膜型BCS的隔膜可能为下腔静脉血栓吸收机化后所致。目前的各种人工血管转流手术远期疗效不佳,应推广介入治疗和病变的根治性切除术,而过去分型中的下腔静脉狭窄实际是因肝脏增大压迫所致,不应该包括在BCS分型中。     

布-加综合征和肝内型门静脉高压症患者血流动力学比较

目的:探讨布-加综合征(Budd-Chiarisyndrome,B-CS)及肝内型门静脉高压症(portalhypertension,PHT)患者血流动力学变化之间的差异。方法:利用彩色多普勒超声对40例B-CS患者、24例PHT患者和24名正常人的门静脉(PV)内径和平均血流速度、每min血流量(Q)进行测算,同时观察肝静脉(HV)、下腔静脉(IVC)变化和肝内侧支血管的形成情况等。以24例健康者为正常对照。结果:B-CS组及PHT组PV内径均大于正常对照组(P均<0.05),PV平均血流速度和Q均小于正常对照组(P均<0.05)。B-CS组上述3指标均小于PHT组(P均<0.05)。B-CS组患者HV病变38例(95%),表现为开口闭塞或狭窄,远端扩张、迂曲等;PHT组发现HV轻度受压狭窄10例(41.7%),HV显示不清3例(12.5%);正常对照组HV均显示正常。IVC病变24例(60%),主要表现为IVC膜性阻塞、狭窄、闭塞,阻塞下方有血栓和IVC扩张,IVC壁搏动消失,血流速度加快、呈双向和逆流等;PHT组发现IVC轻度狭窄8例(33.3%),余正常;正常对照组IVC显示正常。B-CS组特征性的肝内侧支血管形成,表现为HV之间的交通支开放,副肝静脉扩张等;PHT组和正常对照组未发现肝内侧支。结论:B-CS涉及多支HV、PV和IVC系统,患者存在复杂的血流动力学变化。肝内侧支出现是B-CS区别于PHT的特征性表现。     

Etiology, treatment, and classification of Budd-Chiari syndrome

Since Professor WANG Zhong-gao＇s creative work on thesystemic treatment of the Budd-Chiari syndrome（BCS）, this debilitating disease has been more and more widely recognized in China. Several large-scale studies of surgery or intervention strategies for treating BCS have been reported. However, much controversy still remains regarding many aspects of this disease, including its etiology, treatment, and classification. This review explores these controversies with emphasis on areas that merit further study.     

肠系膜上静脉-下腔静脉C形架桥联合门-奇断流术治疗肝静脉闭塞型布-加综合征

目的；探讨一种新的治疗肝静脉闭塞型布-加综合征的手术方式，方法：对88例肝静脉闭塞型布-加综合征患者采用人工血管肠系膜上静脉-下腔静脉C形架桥联合门-奇断流术，脾动脉结扎或脾大部切除术进行治疗，结果：所有患者均顺利完成手术，分流前平均门静脉压为36cmH2O(31-45cmH2O,1cmH2O=0.00098kPa),分流后为26cmH2O(21-33cmH2O)。平均下降10cmH2O。3例死于术后并发症；8例发生乳糜漏，于术后5d-3个月恢复正常。74例随访0.5-7a，无肝性脑病和复发出血，腹水消失61例，明显减少13例，彩超检查人工血管均通畅。结论：此手术方式是治疗肝静脉闭塞型布-加综合征较简单，有效的术式。     

Budd－Chiari综合征的外科治疗

手术是治疗Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的唯一方法。１９９０年１０月～１９９２年２月我科收治１０例，其中行下腔静脉－右心房分流水及门静脉－右心房分流术各４例，２例因术中发现肠系膜上静脉很细、下腔静脉压力正常而放弃手术。术后存活５例，死亡３例。肝包囊虫病可导致此病；人造血管走行经肝前优于经肝后；病人年龄超过５０岁，手术应慎重；病因为肝炎者术后佐复较差；围手术期对心脏及肾脏功能应予以密切关注。     

基于布-加综合征的下腔静脉血管三维重建

目的 利用布-加综合征患者的MR影像数据建立个体化的下腔静脉血管三维数字化模型,探讨其意义以及对临床的应用价值.方法 对典型布-加综合征患者进行MR增强扫描,采集其下腔静脉影像数据集,将采集到的DICOM格式的数据导入Mimics 10.01软件,运用阈值分割技术对下腔静脉血管进行分割,利用3D计算功能对其重建.结果 所建个体化下腔静脉血管三维模型符合临床解剖特性,可以任意缩放、任意角度旋转,显示血管空间结构,并可以进行距离、角度以及狭窄度的测定.结论 获得了布-加综合征患者的个体化下腔静脉血管三维模型.该模型可为人体解剖以及医学影像学教学提供形态学参考.     

Preliminary evaluation of magnetic resonance fresh blood imaging for diagnosis of Budd-Chiari syndrome

Background Budd-Chiari syndrome （BCS） is a rare disease with portal hypertension caused by the blockage of the hepatic vein and/or the inferior vena cava （IVC）. Angiography is the ＂golden standard＂ for diagnosis, but it is an invasive examination. To assess the diagnostic value of a fresh blood imaging （FBI） relative to BCS, we used a magnetic resonance angiography （MRA） with an FBI sequence for a preoperative evaluation of the BCS patients in this study. Methods Fifty patients who were suspected of having BCS after they had been checked by a B-ultrasound were studied. 2D and 3D FBI were performed on a 1.5T superconductive MR scanner. Original images were rebuilt using a maximal intensity projection （MIP） method on the console. Two doctors reviewed all images before they learned of the angiography results. We then compared the diagnoses obtained from the FBI and angiography results to evaluate the diagnostic value of the FBI. Results Forty-one patients were diagnosed as BCS and 9 as non-BCS based on an angiography. The FBI correctly diagnosed 38 patients, incorrectly diagnosed 1 patient, and missed diagnosis in 3 patients. Thus, the diagnostic sensitivity of the FBI is 93% （38/41）, the specificity is 89% （8/9） and the accuracy is 92% （46/50）. The FBI images of the 13 membranous stenoses of the IVC showed a sudden stenosis of the post-liver segment of the IVC. The Images of the 5 patients with a membranous obstruction of the IVC showed IVC thickening and an absence of blood signals in the post-hepatic segment of the IVC. The images of the 4 patients with the segmental thrombosis of the IVC showed abnormal and intermittent signals in the IVC. The images of the 6 patients with a simple hepatic vein obstruction showed obstructive hepatic veins. The images of the 6 patients with the stenosis of both the IVC and the hepatic veins showed the stenosis of the IVC, the thickening of the hepatic veins and the formation of a compensatory circulation within the liver. Lastly, the images of the 7 patients showed a combination of the IVC thrombosis with stenosis or with the obstruction of one or two hepatic veins. Conclusions An FBI can show a membranous stenosis, and an obstruction and thrombosis of the IVC. In addition, it can also demonstrate the thickening of the flexural hepatic vein and the development of intra-hepatic compensatory branches with slow blood flow. Thus, it can guide the puncturing and opening of the hepatic vein involved in an interventional therapy for BCS patients.