布—加综合征的介入治疗

随着彩色超声、CT、MRI及血管造影术等诊断方法的普及 ,目前介入治疗已经成为治疗 BCS的主要方法之一。与外科治疗方法相比 ,介入治疗具有创伤小 ,效果确切、肯定等优点。介入方法治疗 B- CS是近年来的重大进展 ,主要方法有经皮经腔血管成形术 (PTA)支架植入术 (ES)、经颈静脉肝内门体分流术 (TIPS)、经皮肝穿刺肝静脉球囊扩张术和经皮经腔激光血管成形术。介入治疗的主要目的是开通狭窄或闭塞的下腔静脉或 (和 )肝静脉流出道或 (和 )创建新的流出道 ,使升高的下腔静脉或 (和 )门静脉压力恢复正常。 1974年 ,日本学者 Equchi首先采…     

138例肝静脉阻塞型Budd-Chiari综合征介入治疗的研究

背景肝静脉阻塞型Budd-Chiari综合征(BCS)的病因尚未完全明了,其传统治疗方法为门体或肠腔分流术,近年介入放射学技术已成为治疗BCS的常用方法.目的总结11年期间以介入技术治疗肝静脉阻塞型BCS的经验,评价其疗效.方法以介入技术治疗138例肝静脉阻塞型BCS患者.介入治疗方法有经皮腔内血管成形术(PTA)、置入支架和经导管清除血栓.以血管造影表现和压力变化评价即刻疗效,以症状显著改善或消除评价临床疗效,随访期间行超声检查,部分复查CT和CT血管造影(CTA).结果肝静脉阻塞多为膜性阻塞(60.1%).同时存在肝静脉和下腔静脉阻塞者占25.4%,合并下腔静脉血栓占8.0%,肝静脉血栓形成占5.1%.介入治疗成功率为97.1%.单纯行肝静脉球囊扩张成形术28例(20.9%),球囊扩张后向狭窄段置入支架106例(79.1%).介入开通阻塞后,肝静脉压力从(36±9)cm H2O(1 cm H2O=0.098 kPa)降至(18±7)cm H2O.术中未出现并发症.治疗成功者术后相关症状明显改善或完全消失;32例术前以门静脉高压症为主要表现者术后未发生静脉曲张破裂出血,复查内镜示静脉曲张程度减轻.术后随访118例,均生存,其中9例(7.6%)临床症状复发,经血管造影证实支架区狭窄,开通再狭窄成功6例.结论介入放射学技术,如PTA、置入支架和经导管清除血栓是治疗肝静脉阻塞型BCS安全和有效的方法,远期疗效优良.     

球囊扩张合并支架置入治疗肝静脉阻塞型布-加综合征疗效观察

目的 观察球囊扩张合并支架置入治疗单纯肝静脉阻塞型布-加综合征(Budd-Chiari-syndrome,BCS)的疗效.方法 2001年7月至2006年9月采用多普勒超声引导下经皮经肝穿刺肝静脉并肝静脉造影术、经颈静脉行肝静脉成形术、肝静脉球囊扩张及支架置入术治疗肝静脉阻塞型BCS患者43例.结果 术前43例肝静脉平均压力为32.5 cm H2O(1 cm H2O=0.098 kPa),支架置入后立刻下降为20 cm H2O(t=11.5,P＜0.01),再次肝静脉造影显示肝静脉支架通畅,肝内肝静脉侧支消失.其中38例症状立刻消失,5例部分好转.术后43例随访期间(1～62个月,平均31.5个月),1例出院后1个月因上消化道大出血死亡,其余42例症状体征均无再发,术后多普勒超声检查肝静脉均通畅.治疗成功率为100%,所有患者均未出现严重并发症.结论 多普勒超声引导下经皮经肝穿刺肝静脉和经颈静脉途径开通肝静脉,球囊扩张成形及支架置入治疗单纯肝静脉阻塞型BCS安全、有效.     

经颈静脉肝内门体分流术对酒精性肝硬化导致门静脉高压的治疗效果与安全性分析

目的分析经颈静脉肝内门体分流术(TIPS)治疗酒精性肝硬化导致门静脉高压的有效性及安全性。方法回顾性总结2006年6月至2011年6月本院30例接受TIPS治疗的酒精性肝硬化导致门静脉高压的患者资料,记录术前及术后门静脉压力、腹水、脾功能亢进、肝功能等指标。随访终点为术后2 a,观察术后并发症包括消化道再出血、支架堵塞、腹水及肝性脑病发生情况,并分析肝性脑病发生与患者临床参数的关系。穿刺前、后门静脉压力差别采用配对t检验分析;Kaplan-Meier方法用于术后临床参数与肝性脑病发生相关性分析。结果 TIPS手术成功率为100%(30/30),门静脉压力术前(37.27±2.52)cm H2O降为术后(24.6±2.58)cm H2O,差异具有统计学意义(P0.05)。术后2 a内,消化道再出血率为3.3%(1/30);腹水治疗有效率达88.9%(16/18);支架狭窄发生率为6.7%(2/30);肝性脑病发生率为40%(12/30)。Kaplan-Meier分析发现患者术前Child-Pugh分级与术后肝性脑病发生密切相关(P=0.04)。结论 TIPS是治疗酒精性肝硬化门静脉高压相关并发症安全有效的微创方法,术前ChildPugh分级是影响患者肝性脑病发生的重要因素。     

经颈静脉肝内门体分流术后支架功能障碍的原因分析及处理策略

目的探讨经颈静脉肝内门体分流术(TIPS)术后COOK裸支架、Wallgraft覆膜支架和Fluency覆膜支架3种血管支架出现功能障碍的原因及处理策略。方法收集2011年1月-2017年7月于西部战区总医院随访的支架功能障碍并行TIPS修正术患者54例的临床资料。术中通过造影及门静脉压力的情况,选择球囊扩张术、同轴支架置入术和平行TIPS术修正支架功能障碍。采用Kruskal-Wallis H检验比较3种支架狭窄或闭塞时间,采用配对t检验比较修正前后PVP。通过影像学资料判断支架功能障碍情况,分析支架狭窄或闭塞的原因。结果 54例患者全部成功实施TIPS修正,狭窄部位分别发生在肝静脉段、肝实质或门静脉段,高发时间窗为术后6～24个月。COOK支架、Wallgraft支架、Fluency支架狭窄或闭塞中位时间分别为17. 0、10. 0、17. 0个月。COOK支架和Fluency支架发生狭窄或闭塞时间晚于Wallgraft支架(P值分别为0. 013、0. 023),COOK支架和Fluency支架发生狭窄或闭塞时间差异无统计学意义(P=0. 893)。应用修正术式方面,球囊扩张4例,同轴支架置入39例,平行TIPS 11例,所有患者术后门静脉压力明显低于术前,差异有统计学意义[(25. 6±4. 8) cm H_2O vs (34. 7±6. 4) cm H_2O,P 0. 001]。结论 TIPS术后Wallgraft支架功能障碍时间明显早于COOK祼支架、Fluency支架,提示Wallgraft支架不适用于TIPS分流道的初始创建。术中根据具体状况选择适宜的修正术式,均能够修复支架功能障碍,恢复分流道通畅。     

造血干细胞移植与肝(小)静脉闭塞病

文献报道的肝静脉闭塞病（HVOD）通常是指肝小叶中央静脉和小叶下静脉损伤,导致管腔狭窄或闭塞,实际上应称肝小静脉闭塞病。本病主要累及肝内小静脉,有别于BuddChiari综合征（BCS）,后者主要是肝静脉及其属支狭窄或阻塞,部分累及肝段下腔静脉。HVOD及BCS均可引起窦后性门静脉高压症、肝细胞坏死和肝功能减损。曾不断有草药土三七引起HVOD的报道。近年报道HVOD主要发生于造血干细胞移植后,发生率最多可达54％,严重程度差别很大,重者死亡率达67％,早诊早治是改善预后的关键。     

经颈内静脉肝内门体分流术治疗肝硬化门脉高压症56例

目的:探讨经颈内静脉肝内门体分流术 (transjugular intrahepatic portosystemic shunt, TIPS)治疗肝硬化门脉高压症的临床疗效及并发症．方法:肝硬化并发门脉高压症患者56例,行 TIPS术,支架置入前后测量门静脉主干压力, 测定门静脉、脾静脉内径,测定门静脉、分流道血流速度;检测外周血象．结果:TIPS术成功率100％,门脉主干压 (cmH2O)24．5±4．2(P<0．01 vs 术前的45．8± 3．6),门静脉内径及脾静脉内径(cm)分别为 1．28±0．095和0．96±0．064(P<0．01 vs术前的 1．62±0．074和1．28±0．032),门脉主干血流速度(cm／s)48．0±17．6(P<0．01 vs术前的13．2± 3．5),分流道血流速度(cm／s)164．0±58．8．脾功能亢进患者30例,WBC(×109／L),PLT(× 109/L),HGB(×g／L)分别为3．92±0．76,80．74± 16．82,118．20±10．24(P<0．01 vs术前的2．65± 0．58,42．56±12．34,86．52±12．68)．食道胃底静脉曲张,腹水等临床症状明显好转．常见并发症有肝性脑病,支架狭窄及闭塞．结论:TIPS是一种治疗肝硬化门脉高压症的有效方法,他能有效地降低门脉压,控制食道、胃底静脉曲张破裂出血．     

Budd-Chiari综合征、肝小静脉闭塞病与肝硬化的鉴别

Budd-Chiafi综合征（BCS）是由肝静脉或其开口以上的下腔静脉阻塞性病变引起的以下腔静脉阻塞、门静脉高压为特点的综合征，诊断依靠肝静脉血管造影，治疗重点是解除梗阻。肝小静脉闭塞病是肝小叶中央静脉和小叶下静脉损伤导致管腔狭窄或闭塞而产生的门静脉高压症，临床表现类似于BCS，诊断依靠肝组织活检。这两种疾病的治疗和预后均与肝实质性病变导致的肝硬化不同．临床上需要注意对这三种疾病的鉴别。     

经颈静脉肝内门体静脉分流术应用近况

经颈静脉肝内门体静脉分流术(Tran-sjugular Intrahepatic Portosystemic Shunt,TIPS)是近几年发展起来的一项新的介入治疗技术。是采用特定的穿刺、扩张器械及血管内支架,在肝内的肝静脉和门静脉大分支之间建立一分流通道,使门静脉血经此通道流入下腔静脉,从而降低门静脉压。     

影像学评价对选择Budd-Chiari综合征介入治疗途径的价值

目的 探讨BCS介入术前彩色多普勒超声、CT、磁共振、血管造影等影像学检查对设计介入治疗途径的价值。方法 根据彩色多普勒超声、CT、磁共振、血管造影等影像学检查结果，依据下腔静脉与肝静脉、副肝静脉的关系．采用不同的介入手术方法及入路，经股静脉、经颈静脉、经皮经肝穿刺或经副肝静脉开通下腔静脉、肝静脉、副肝静脉或同时开通。结果 根据术前影像学检查，决定手术方案、选择合理的术式和入路。50例手术均获得成功，疗效确切。其中下腔静脉狭窄34例（膜性20例，节段性14例），肝静脉狭窄、闭塞10例，下腔静脉狭窄伴肝静脉阻塞3例，肝静脉闭塞伴副肝静脉狭窄、闭塞2例。49例下腔或，和肝静脉开通后球囊扩张，共置入金属支架29例。肝小静脉闭塞1例，行TIPS术。术后随访1～36月，其中2例下腔静脉膜性狭窄球囊扩张术后分别于6和8个月出现再狭窄，后行金属支架置入．症状消失。1例因肾功能衰竭死亡。结论 通过术前检查．正确选择手术方式，可避免手术盲目性，避免再次手术，减少手术并发症，节约手术费用，获得良好的手术效果。     

Is there an Alternative to TIPS? Ultrasound-guided Direct Intrahepatic Portosystemic Shunt Placement in Budd-Chiari Syndrome

Budd-Chiari syndrome is a spectrum of manifestations which develops as a result of hepatic venous outflow obstruction. Transjugular intrahepatic portosystemic shunt (TIPS) is a minimally invasive vascular and interventional radiological procedure indicated in the management of refractory ascites in such patients. Conventional TIPS requires the presence of a patent hepatic vein and reasonable accessibility to the portal vein, and in patients with totally occluded hepatic veins, this procedure is technically challenging. Direct intrahepatic portosystemic shunt (DIPS) or so called “percutaneous TIPS” involves ultrasound-guided percutaneous simultaneous puncture of the portal vein and inferior vena cava followed by introduction of a guidewire through the portal vein into the inferior vena cava, as a deviation from conventional TIPS. Described here is our experience with DIPS. Three patients with BCS who had refractory ascites but were unsuitable for conventional TIPS due to occlusion of the hepatic veins were chosen to undergo the DIPS procedure. Our technical success was 100%. The shunts placed in two patients remain patent to date, while the shunt in a third patient with underlying antiphospholipid syndrome was occluded a month after the procedure. The percutaneous TIPS procedure seems to be technically feasible and effective in the management of refractory ascites as a result of BCS, particularly in the setting of occluded hepatic veins.     

Direct intrahepatic cavo-portal shunts in Budd-Chiari syndrome: Role of simultaneous fluoroscopy and trans-abdominal ultrasonography.

BACKGROUND: Transjugular intrahepatic porto-systemic shunt (TIPS) for Budd-Chiari syndrome (BCS) can be inserted from inferior vena cava or hepatic vein to portal vein. The former is performed when hepatic veins are not suitable and is technically more challenging. METHODS: In this retrospective study, 7 patients with chronic BCS needed cavo-portal shunt as hepatic veins were neither amenable to plasty nor provided access for TIPS placement. Simultaneous fluoroscopic and trans-abdominal ultrasound guidance was used at the time of portal vein puncture. RESULTS: Technical success and clinical improvement were obtained in all patients. Median 3 (range 1-4) attempts were needed to puncture the portal vein. There were no significant complications. Uncovered stents were used in six patients and stent occlusion was common, but could be managed by re-intervention. CONCLUSION: Cavo-portal shunt is an effective technique for patients with BCS uncontrolled by medical therapy. Additional trans-abdominal ultrasound in oblique parasagittal plane keeps the procedure safe.     

TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy.

Patients with Budd-Chiari syndrome (BCS) may require treatment with portal decompressive surgery or liver transplantation. Transjugular intrahepatic portosystemic shunt (TIPS) represents a new treatment alternative, but its long-term effect on BCS outcome has not been evaluated. Twenty-one patients with BCS consecutively admitted to our unit were evaluated. The mean follow-up was 4 +/- 3 years. Seven patients had nonprogressive forms and were successfully controlled with medical therapy; 1 case, with a short-length hepatic vein stenosis was successfully treated by angioplasty. All 8 patients are alive and asymptomatic. The remaining 13 patients, had a TIPS because of clinical deterioration (in one of them, because early TIPS thrombosis a successful side-to-side portacaval shunt [SSPCS] was performed) followed by an improvement in clinical condition. However, a patient with fulminant liver failure before TIPS insertion, died 4 months later and another patient with cirrhosis at diagnosis had liver transplantation 2 years later. The remaining 11 patients are alive and free of ascites. In 3 of these patients TIPS is patent after 3, 6, and 12 months. The remaining 8 patients developed late TIPS dysfunction. In two of these cases, after angioplasty and restenting, TIPS is patent after a follow-up of 9 and 80 months. In 5 other patients, recurring TIPS occlusion was not further corrected because no signs of portal hypertension were present. In conclusion, in patients with BCS uncontrolled with medical therapy, TIPS is a highly effective technique that is associated with long-term survival.     

肝硬变门脉高压新疗法：经颈静脉肝内门－腔静脉内支架分流术──附36例分析

经颈静脉肝内门－腔静脉内支架分流术（ＴＩＰＳ）是用非外科手段治疗肝硬变、门脉高压，胃、食管静脉曲张的新技术，具有创伤小，技术成功率高，并发症少的优点。本文报告了３６例（包括３例急诊ＴＩＰＳ），成功率为９４．４４％（３４／３６）。分流道的直径为８－１０毫米。术后平均门脉压力从术前的４１．６７ｃｍＨ２Ｏ降至２４．８９ｃｍＨ２Ｏ，绝大多数患者腹水吸收，静脉曲张减轻或消失。我们的初步经验：ＴＩＰＳ能降低门脉压，控制静脉曲张破裂出血，消除腹水。是一种安全、有效的方法。ＴＩＰＳ的长期效果有待进一步观察。     

Transjugular intrahepatic portosystemic shunt by direct transcaval approach in patients with acute and hyperacute Budd-Chiari syndrome

When Budd-Chiari syndrome (BCS) is due to occlusion of all three hepatic veins, the standard transjugular intrahepatic portosystemic shunt (TIPS) technique can be extremely laborious. A feasible alternative is to use the direct transcaval approach, by which a shunt can be created directly between the intrahepatic inferior vena cava and the portal vein. We describe two patients (one with acute BCS and one with hyperacute BCS) who were successfully managed with this modified technique. Both patients recovered; one of them underwent elective liver transplantation 15 months after the procedure, whereas the other still had good hepatic function and a patent stent 24 months after the procedure. We conclude that, in selected patients with acute and hyperacute BCS, placement of a TIPS by the direct transcaval approach is a rapid and effective emergency procedure, which can either be curative or function as a bridge for elective liver transplantation.     

脾腔小口径分流联合断流术对肝硬化门静脉高压症患者肝血流动力学和储备功能的影响*

目的观察脾腔小口径分流联合断流术对肝硬化门静脉高压症患者肝血流动力学和肝储备功能的影响。方法86例肝硬化门静脉高压症患者,分别行脾腔小口径分流联合断流术（联合组,58例）和贲门周围血管离断术（断流组,28例）。通过术中自由门静脉压力（FPP）监测和术后彩色多普勒超声检查明确不同手术方式对门静脉血流量（PVF）的影响,通过吲跺氰绿排泄试验（ICGR15）观察肝储备功能和有效肝血流量（FHF）的变化。结果联合组术后FPP、PVF、FHF和ICGR15分别为（31.4±2.4） cmH₂O、（900±350）ml/min、（551±246）ml/min和（31.2±13.8）%,较术前有明显降低或升高【（38.2±3.6） cmH₂O、（1250±360）ml/min、（696±300）ml/min和（23.6±11.9）%,P<0.05】。断流组术后FPP、PVF,FHF和ICGR15为（32.8±3.2） cmH2O、（980±250）ml/min、（507±140）ml/min和（27.4±13.0）%,也较术前明显升高或降低【（36.9±3.9） cmH2O、（1320±320）ml/min）、625±158）ml/min和（22.2±13.4）%,P<0.05】。与断流组比较,联合组FPP下降更为明显（P<0.05）,而PVF、FHF和ICGR15比较无显著性差异（P>0.05）。术后30天联合组肝功能良好、轻度和重度代偿不全发生率为72.4%、19.0%和8.6%,断流组分别为67.9%、21.4%和10.7%,两者差异无显著统计学意义。随访5~36月,联合组分别有1例和4例发生上消化道出血和肝性脑病,断流组分别为3例和2例。结论脾腔小口径分流联合断流术治疗门静脉高压症是安全有效的,该术式在降低门静脉压力同时,可维持门静脉向肝血流,保护肝脏储备功能,防止肝功恶化衰竭。     

布-加综合征、肝血窦阻塞综合征与肝硬化的鉴别

布-加综合征是由肝静脉或其开口以上的下腔静脉阻塞性病变引起的以下腔静脉阻塞、门静脉高压为特点的综合征,诊断依靠影像学检查,治疗的关键是解除梗阻。肝血窦阻塞综合征是指肝窦内皮完整性破坏和肝窦内充血阻塞而产生的肝内窦后性门静脉高压症,临床表现类似布-加综合征,诊断依靠肝组织活检。这两种疾病的治疗和预后均与肝实质病变导致的肝硬化不同,所以临床上需要注意这三种疾病的鉴别。     

肝硬化食管胃底静脉曲张破裂出血的急诊TIPS术可行性

目的探讨经颈静脉肝内门体分流术(transjugular intrahepatic portosystemic shunt,TIPS)用于急性肝硬化食管胃静脉曲张破裂出血的可行性。方法选择2011年9月至2013年9月收治的28例急性肝硬化食管胃静脉曲张破裂出血患者,均实施急诊TIPS术。结果 28例患者均1次操作成功接受TIPS术,术后24 h的止血率为100.0%。患者的平均门静脉压力术后较术前显著下降,分别为(27.01±5.32)和(38.23±7.41)cmH2O,门体循环压力差显著减小,分别为(18.76±4.70)和(30.45±7.69)cmH2O,经比较差异均有统计学意义(t=9.56,8.74,均P0.05)。术后,患者肝功能各项指标及凝血酶原时间较之术前均未出现显著改变,经比较差异均无统计学意义,(均P0.05)。术后随访3个月,所有患者支架均通畅,1例分流道为门静脉左支患者出现狭窄闭塞。1例患者出现肝性脑病,1例患者出现肝功能衰竭,经治疗无效死亡。结论急诊TIPS术用于急性肝硬化食管胃静脉曲张破裂出血可以获得良好的效果,安全性高。