直视下Budd-Chiari综合征根治术术式选择

目的：探讨Ｂｕｄｄ－Ｃｈａｉｒｉ综合征（ＢＣＳ）４种根治术的适应证及其优缺点。方法：１９９３年１１月～２０００年１２月对１１４例ＢＣＳ病人行直视下根治术,其中单纯隔膜切除２０例,下腔静脉病变段切开、心包片成形３９例,下腔静脉病变段切除、人工血管原位移植４２例,肝静脉主干闭塞段及其上方的下腔静脉闭塞段切除、肝静脉流出道扩大成形、肝静脉开口至下腔静脉的右房入口处人工血管原位移植术１３例。结果：全组无手术死亡。住院期间４例死于并发症。随访６～８４个月,复发５例,２例无效,均为心包片扩大成形术后。结论：４种根治术有各自不同的适应证。合理的术式选择是取得良好远期疗效的关键。     

布-加综合征根治术后复发的治疗

目的 分析布-加综合征根治术后复发的原因及处理.方法 回顾性分析102例布加征根治术后下腔静脉和(或)肝静脉再狭窄或闭塞34例患者的临床资料,统计下腔静脉及肝静脉通畅率,探讨术后复发原因,采用生存分析法研究复发危险因素.结果 102例布-加综合征患者根治术后34例出现再狭窄或闭塞,其中32例为下腔静脉病变(其中19例患者同时合并肝静脉病变),2例患者为肝静脉病变.对于复发患者的治疗:1例行二次根治术,16例行下腔静脉和(或)肝静脉球囊扩张,3例行下腔静脉支架植入,保守治疗14例.结论 布-加综合征根治术后具有较高的复发率,复发者可再次行根治术,也可行下腔静脉或肝静脉球囊扩张或支架成形术及药物治疗.复发原因多为下腔静脉血栓形成,肝尾叶外压,瘢痕挛缩等.复发的危险因素有合并高凝因素、术后抗凝疗程多不足半年.     

肝静脉球囊扩张、支架置入术治疗下腔静脉长段闭塞型布加综合征

目的 探讨肝静脉球囊扩张支架置入术治疗下腔静脉长段闭塞型布加综合征( Budd-Chiari syndrome,BCS)的意义.方法 对40例下腔静脉长段闭塞型BCS经彩超、CT、MR对肝静脉情况进行评估后,先经颈静脉行膈上段下腔静脉造影,观察有无肝静脉开口,再用椎动脉导管结合超滑导丝寻找肝静脉;显示肝静脉后,行肝静脉球扩、支架术.结果 40例BCS中,29例为肝静脉开口处隔膜或主干的短段闭塞,其中隔膜5例、主干短段闭塞24例,成功地对28例进行了肝静脉的介入治疗,其中单纯球扩5例,球扩加支架23例,1例穿刺失败.另11例为肝静脉的广泛阻塞无法行肝静脉的介入治疗.随访26例,平均随访(24.0±1.3)个月,症状复发5例(19.2％),彩超见肝静脉再狭窄或闭塞6例(23％).结论 下腔静脉长段闭塞,多数肝静脉病变仅是开口处隔膜或主干的短段闭塞,采用颈静脉入路行肝静脉球扩支架的方法,可以解除肝静脉梗阻、缓解门静脉高压.     

布加综合征根治术后复发再手术11例分析

目的探讨布加综合征(Budd Chiarisyndrome,BCS)根治术的复发原因及再次手术的要点。方法11例根治术复发患者,再次造影,采用下腔静脉病变段切除、人工血管原位移植4例,隔膜切除、取栓2例,隔膜切除取栓、人工血管片扩大成形4例,下腔静脉狭窄部位切开、右肝静脉主干成形、人工血管片做下腔静脉扩大成形1例。结果全组无一例死亡,术后急性心衰2例、胸腔大出血2例,均保守治愈。出院时所有患者症状消失或明显好转。随访6~68个月,平均32个月,效果优良9例,良好2例。结论病变远端下腔静脉游离长度、血栓是否清除干净、扩大成形的材料及大小等因素均会影响根治的远期效果。     

肠系膜上-下腔静脉吻合联合脾动脉结扎、食管胃底周围血管离断治疗II型布-加综合征

目的探讨一种新的治疗下腔静脉无病变或长节段狭窄、肝静脉闭塞的手术方法。方法采用肠系膜上静脉-下腔静脉联合脾动脉结扎、食管胃底周围血管离断术治疗44例下腔静脉无病变或长节段狭窄、肝静脉闭塞型布-加综合征患者。结果44例均顺利完成手术。分流前平均门静脉压为36cmH2O(31～45cmH2O,1cmH2O=0.0098kPa),分流后门静脉压力26cmH2O(21～33cmH2O),平均下降10cmH2O。术后1例死于肝衰竭;1例死于多脏器衰竭;2例有轻度肝性脑病,经对症治疗后好转;4例发生乳糜漏,分别于术后7d至3.5个月自行消失。39例(88.6%),随访0.5～7年。无再出血及肝性脑病发生。腹水消失31例,明显减少7例。经彩色多普勒检查,39例人造血管均通畅。结论此手术方式是治疗下腔静脉无病变或长节段狭窄、肝静脉闭塞型布-加综合征较简单而有效的术式。     

肠系膜上静脉-下腔静脉-下腔静脉人工血管"Y"型架桥术治疗混合型布-加综合征32例分析

下腔静脉-右心房转流术(腔-房转流术)是治疗节段闭塞型布-加综合征(Budd-Chiari syndrome,B-CS)的常用方法,适用于肝后下腔静脉近右房处闭塞式狭窄、病变超过1 cm、至少有一支肝静脉(包括增粗的肝右后静脉)开口于阻塞段下方的下腔静脉并血流通畅的患者.     

肠系膜上-下腔静脉吻合联合脾动脉结扎、食管胃底周围血管离断治疗II型布-加综合征

目的探讨一种新的治疗下腔静脉无病变或长节段狭窄、肝静脉闭塞的手术方法。方法采用肠系膜上静脉-下腔静脉联合脾动脉结扎、食管胃底周围血管离断术治疗44例下腔静脉无病变或长节段狭窄、肝静脉闭塞型布-加综合征患者。结果44例均顺利完成手术。分流前平均门静脉压为36cmH2O(31～45cmH2O，1cmH2O=0．0098kPa)，分流后门静脉压力26cmH2O(21～33cmH2O)，平均下降10cmH2O。术后1例死于肝衰竭；1例死于多脏器衰竭；2例有轻度肝性脑病，经对症治疗后好转；4例发生乳糜漏，分别于术后7d至3．5个月自行消失。39例(88．6％)，随访0．5—7年。无再出血及肝性脑病发生。腹水消失31例，明显减少7例：经彩色多普勒检查，39例人造血管均通畅。结论此手术方式是治疗下腔静脉无病变或长节段狭窄、肝静脉闭塞型布一加综合征较简单而有效的术式。     

肠-腔-房转流术治疗混合型布加综合征的疗效分析

目的 探讨应用人工血管行肠-腔-房转流术(MCAS)治疗混合型布加综合征的临床效果.方法 回顾性分析2000年2月至2004年5月山东大学齐鲁医院收治的17例混合型布加综合征患者的临床资料.17例患者均为全部或两支肝静脉主干阻塞且肝后下腔静脉长段血栓形成或长段闭塞(或)狭窄,应用人工血管行MACS.观察并比较患者手术前后的临床症状、门静脉及下腔静脉压力;患者术后并发症发生率;人工血管通畅率.采用Kaplan-Meier法分析患者生存率,χ2检验和t检验分析相关数据.结果 全组患者无围手术期死亡.17例患者中15例临床症状消失或缓解,与术前比较,差异有统计学意义(χ2=9.78,P<0.05);3例出现并发症;门静脉及下腔静脉压力术后较术前平均下降1.2 cm H2O(1 cm H2O=0.098 kPa)和18.5 cm H2O,其差异有统计学意义(t=2.38,3.06,P<0.05);1、3、5年生存率分别为16/17、15/17、14/17;5年人工血管通畅率为14/17.结论 MCAS可同时缓解混合型布加综合征的门静脉高压和下腔静脉高压,术后降压效果明显,并发症少,5年生存率和人工血管通畅率高,是一种可供选择的手术方式.     

胸部正中切口布加氏综合征根治术远期疗效观察

目的　观察胸部正中切口布加氏综合征根治手术的远期疗效 ,总结根治肝静脉阻塞型布加氏综合征的临床经验 ,探讨一种安全有效的手术方法。方法　 3 0例在不切开胸膜腔和腹腔的情况下 ,经胸部正中切口 ,仅行心包、下腔静脉切开 ,在膈下腹膜外间隙内显出下腔静脉或肝静脉病变 ,在停循环状态下进行局部病灶切除 ,恢复肝脏血液回流 ,当时即见肝脏张力减小、肝脏体积缩小。对存活病例每 6个月随访观察 ,进行肝脏及第二肝门血管彩超或核磁共振检查。结果　 2 9例获得临床治愈 ,随访 1～ 10年 ,均健康存活 ,无并发症 ,无复发 ;1例术后 2周因黄疸、应急性溃疡消化道出血死亡。结论　胸部正中切口是一种比较理想的根治肝静脉阻塞型布加氏综合征的手术入路 ,安全、有效、创伤小、远期效果良好。     

布加综合征合并肝癌十例

目的 探讨布加综合征合并肝癌病人的临床特征、预后及治疗情况.方法 对10例布加综合征合并肝癌病人的临床资料进行回顾性分析.彩色多普勒超声探查或(和)下腔静脉造影和肝静脉造影确诊布加综合征:Ⅰ a型4例,Ⅱ型3例,Ⅲ a型3例.肝细胞癌由彩色多普勒超声、增强螺旋CT和病理检测诊断;肝癌单发8例,多发2例.8例均成功开腹进行肝癌切除术,3例并行肠系膜上静脉-下腔静脉人工血管反C型分流术(肠-腔分流术);5例开腹手术前2～3 d介入行下腔静脉球囊扩张成形术或(和)(副)肝静脉扩张成形术.2例多发肝癌合并布加综合征病人行介入超选择性肝动脉化疗栓塞术+下腔静脉球囊扩张成形术或(和)(副)肝静脉扩张成形术.结果 1例行肠-腔分流术+肝癌切除术病人术后5 d死于肝衰竭;1例行肠-腔分流术病人术后4 d出现乳糜漏,治疗15 d后好转.随访9例,随访时间为术后半年至5年;2例多发肝癌病人介入术后1年和半年死于肝衰竭;2例病人在术后1年和2年死于肝癌复发转移.结论 布加综合征合并肝癌病人预后不良,在解除布加综合征病人肝脏淤血的状态的同时尽早并尽可能的切除肝癌病灶是目前最有效的治疗方法.     

Living Donor Liver Transplantation in Budd-Chiari Syndrome: A Single-Center Experience

Budd-Chiari syndrome (BCS), which is characterized by hepatic venous outflow obstruction due to occlusion of the major hepatic vein and/or the inferior vena cava (IVC), is rare. Traditionally, a caval resection is advocated for these patients; however, such a manenver renders living donor liver transplantation (LDLT) impossible. We encountered BCS in 4/377 LDLT patients during a 5-year period (January 2003 to December 2007). This report examine the various surgical modifications in these 4 patients, who underwent to LDLT for BCS. Resection of right hepatic vein (RHV) with an adjacent fibrotic part of the IVC with direct anastomosis of the graft RHV to the IVC was performed in 2 patients. One patient underwent retrohepatic IVC excision and reconstruction with a cryopreserved autologous IVC graft. The fourth patient, with a preexisting mesoatrial shunt for BCS, underwent conversion of this to a RHV atrial shunt. Graft and patient survivals were 100%. There were few complications in either donors or recipients. LDLT for BCS can be performed safely with adequate venous drainage techniques and with anticoagulant therapy and good follow-up for early diagnosis and treatment of recurrence leading to excellent long-term results.     

A 27-year experience with surgical treatment of Budd-Chiari syndrome

OBJECTIVE: To determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. SUMMARY BACKGROUND DATA: Experiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. METHODS: From 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation. RESULTS: In the 32 patients with BCS resulting from hepatic vein occlusion alone, SSPCS had a surgical death rate of 3%, and 94% of the patients were alive 3.5 to 27 years after surgery. All 31 survivors remained free of ascites and almost all had normal liver function. No patient with a patent shunt had encephalopathy. The SSPCS remained patent in all but one patient. Liver biopsies showed no evidence of congestion or necrosis, and 48% of the biopsies were diagnosed as normal. Mesoatrial shunt was performed in eight patients with BCS caused by IVC thrombosis. All patients survived surgery, but five subsequently developed thrombosis of the synthetic graft and died. Because of the poor results, mesoatrial shunt was abandoned. Instead, a high-flow combination shunt was introduced, consisting of SSPCS combined with a cavoatrial shunt (CAS) through a Gore-Tex graft. There were no surgical or long-term deaths among 10 patients who underwent combined SSPCS and CAS, and the shunts functioned effectively during 4 to 16 years of follow-up. Ten patients with advanced cirrhosis were referred too late to benefit from surgical portal decompression, and they were approved and listed for orthotopic liver transplantation. Three patients died of liver failure while awaiting a transplant, and four patients died after the transplant. The 1- and 5-year survival rates were 40% and 30%, respectively. CONCLUSIONS: SSPCS in BCS with hepatic vein occlusion alone results in reversal of liver damage, correction of hemodynamic disturbances, prolonged survival, and good quality of life when performed early in the course of BCS. Similarly good results are obtained with combined SSPCS and CAS in patients with BCS resulting from IVC occlusion. In contrast, mesoatrial shunt has been discontinued in the authors' program because of an unacceptable incidence of graft thrombosis and death. In patients with advanced cirrhosis from long-standing, untreated BCS, orthotopic liver transplantation is the only hope of relief and results in the salvage of some patients. The key to long survival in BCS is prompt diagnosis and treatment by portal decompression.     

Budd-Chiari 综合征根治术后复发的介入治疗：附15例报告

目的：探讨布加综合征（BCS）根治术后复发患者介入治疗的可行性及疗效。 方法：回顾性分析2007年3月—2013年9月15例BCS根治术后复发行介入治疗患者的临床资料。 结果：15例患者,年龄30~68岁,平均46.4岁;共行18例次介入治疗（1例3次,1例2次）,其中下腔静脉球囊扩张成形术10例次,置管溶栓后下腔静脉球囊扩张成形术5例次,下腔静脉、肝静脉及副肝静脉球囊扩张成形术2例次,下腔静脉球囊扩张成形术并下腔静脉支架植入1例次。围手术期未出现肺动脉栓塞、心包填塞等并发症 。15例患者随访12~106个月,平均41.9个月,13例未出现复发症状及体征,1例肝静脉闭塞在外院行球囊扩张支架成形术,1例下腔静脉再次闭塞保守治疗。 结论：对于BCS根治术后复发患者,选择及时介入治疗安全有效,可以改善累积通畅率及预后。     

Hanging of the Hepatic Veins Septa: A Safe Control Prior and During Outflow Anastomosis in Liver Transplantation

Inferior vena cava (IVC) preservation during orthotopic liver transplantation (OLT) is known as the “piggyback” technique. The end-to-side anastomosis is constructed between the graft's IVC and recipient's hepatic veins using a Satinsky side clamp applied in a transverse position. To stabilize the large Satinsky clamp and preserve a sufficient vascular stump after hepatectomy and before graft implantation, we propose a technical innovation consisting of hanging the septa between the left and middle hepatic vein and between the middle and right hepatic vein using 2 tapes. This technique showed some advantages when performing the caval outflow anastomosis, representing a further technical refinement of the piggyback end-to-side technique for the implantation on the 3 hepatic veins. From November 2001 to September 2012, we performed 272 consecutive OLT at our institution with the piggyback technique using the hanging of the hepatic veins septa in all cases. In conclusion, the hanging of the 3 hepatic veins septa presented in this study represents a simple, safe and reproducible technique for the outflow anastomosis using the piggyback technique.     

Surgical Techniques and Long-Term Outcomes of Living Donor Liver Transplantation for Budd-Chiari Syndrome

We have developed the surgical techniques of living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) and evaluated long-term outcomes including specific complications. BCS is characterized by hepatic outflow obstruction. Liver transplantation from living donors poses a unique challenge as liver replacement therapy does not replace the retrohepatic segment of inferior vena cava (IVC). We have performed 1105 LDLTs in 1055 patients from January 1990 to March 2005. Of these, nine patients (eight males and one female) underwent LDLT for BCS. Five out of nine patients underwent LDLT as a primary procedure and four patients had received other treatments before transplantation. Eight patients presented with chronic and one with fulminant liver failure. Predisposing factors were identified in three patients. IVC reconstruction without patch plasty was performed on four patients. Five patients needed cavoplasty using a replacement vein graft. Of the nine patients, seven are alive at a median follow-up of 58 months (range 1 month to 15.2 years) with two patients developing recurrent hepatic vein stenosis which were treated successfully with metallic stent placement. Two patients died: one from multiorgan failure and the other from pulmonary embolism secondary to disease recurrence. LDLT for BCS is highly effective by using modified cavoplasty and provides good long-term survival which may be obtained by life-long anticoagulant treatment and nonsurgical interventions.     

New Left Lobe Transplantation Procedure with Caval Reconstruction Using an Inverted Composite Graft for Chronic Budd-Chiari Syndrome in Living-Donor Liver Transplantation—A Case Report

When the Budd-Chiari syndrome (BCS) lesion extends to the inferior vena cava (IVC) or the orifices of the hepatic vein, the thickened IVC and/or hepatic vein wall must be removed and IVC reconstruction is required in living-donor liver transplantation (LDLT). In various reports about IVC resection in LDLT for BCS, there are none about left lobe liver transplantation with reconstruction of the retrohepatic IVC (rhIVC). To overcome removal and reconstruction of the rhIVC in LDLT for BCS, we introduced a composite IVC graft that is applicable to both right and left lobe partial liver grafts for LDLT for BCS. Pathogenic IVC was removed together with the native liver between the lower edge of the right atrium and 5 cm above the renal vein junction with the use of venovenous bypass. The e-polytetrafluoroethylene graft was anastomosed to the suprarenal intact IVC. Then the native part was detached at the level of just above the renal junction. The composite graft was inverted and a half rim of the native part of the graft was anastomosed to the posterior wall of the right atrium. Next, the common venous orifice of the left lobe graft was anastomosed to the wall defect which was composed of the anterior wall of the right atrium and the distal end of the native part of the composite graft. In conclusion, our inverted composite graft technique will overcome the weak points of LDLT for BCS, such as incomplete removal of the pathogenic caval wall and reconstruction of the rhIVC.     

Resection and reconstruction of the inferior vena cava for major hepatic resection]

The inferior vena cava (IVC) is partially or segmentally resected in major hepatic resection for malignant hepatic tumors in case of possible direct invasion to the IVC wall or IVC tumor thrombosis. The reconstruction methods of the IVC are divided into three categories depending on the degree of IVC resection: simple suture; patch repair; and segmental replacement. In segmental replacement, a synthetic material such as a cylindrical expanded polytetrafluoroethylene (ePTFE) grafts is widely utilized as a substitute. The total hepatic vascular exclusion technique is usually necessary in concomitant resection of the suprahepatic IVC. When a longer duration of hepatic vascular exclusion is required to resect and reconstruct the suprahepatic IVC and hepatic vein confluence, in situ hypothermic perfusion, the ante situm technique, or ex vivo bench surgery must be applied. When an ePTFE graft is replaced in the resected IVC, a Carrel patch of the IVC is used for the hepatic vein orifice to maintain anastomotic patency. Alternatively, the hepatic vein can be reanastomosed to an inferior vena caval segment transpositioned from the intact infrahepatic IVC portion by replacing the resected infrahepatic IVC with an ePTFE graft.     

No-Touch En Bloc Right Lobe Living-Donor Liver Transplantation with Inferior Vena Cava Replacement for Hepatocellular Carcinoma Close to Retrohepatic Inferior Vena Cava: Case Report

Current studies have shown that living-donor liver transplantation (LDLT) for hepatocelluar carcinoma (HCC) satisfying the Milan criteria does not compromise patient survival or increase HCC recurrence compared with deceased-donor liver transplantation (DDLT). For patients with HCC beyond the Milan criteria, however, worse outcomes are expected after LDLT than after DDLT, despite insufficient data to reach a conclusion. Regarding operative technique, LDLT might be a less optimal cancer operation for HCC located at the hepatic vein confluence and/or paracaval portion. The closeness to the wall of the retrohepatic inferior vena cava (IVC) is greater than in conventional DDLT, rendering it difficult to perform a no-touch en bloc total hepatectomy. An LDLT, which must preserve the native IVC for the piggyback technique during engraftment, may lead to tumor remnants. To reduce recurrences after LDLT, we successfully performed a no-touch en bloc total hepatectomy including the retrohepatic IVC and all 3 hepatic veins. IVC replacement with an artificial vascular graft together with a modified right-lobe LDLT was performed for a patient having advanced HCC close to the hepatic vein confluence and paracaval portion. There was no artificial vascular graft-related complication, such as thrombosis or infection. Despite the limitations of LDLT, requiring the piggyback technique for graft implantation, IVC replacement using an artificial graft led us to perform a no-touch en bloc total hepatectomy as with a conventional DDLT.     

肝静脉阻塞型布加综合征的腔内治疗

目的 总结肝静脉阻塞型布加综合征(Budd-Chiari syndrome,BCS)的腔内治疗经验.方法 回顾性分析32例肝静脉阻塞型BCS的临床资料.分别行下腔静脉球囊扩张成形或支架植入术+脾肾静脉分流术;经股静脉或颈静脉入路肝静脉成形术和经皮肝穿刺肝静脉联合颈静脉和/或股静脉入路肝静脉成形术或支架植入术.结果 2例行经皮肝穿刺肝静脉造影时未发现主肝静脉而放弃治疗,其余病例均成功行肝静脉成形和下腔静脉成形术.肝静脉扩张成形前后测肝静脉压力由术前(43±8)cm H_2O降至术后(16±4)cm H_2O(t=21.23,P<0.01).术后1周原有症状明显缓解,腹水消失,腹胀减轻,胸腹壁曲张静脉塌陷.围手术期发生2例穿刺针道出血,经剖腹止血后痊愈.本组随访25例,随访率78.1%.随访时间5～65个月,平均(26.0±2.0)个月.无支架移位及肝静脉再狭窄或闭塞,胸腹擘曲张静脉消失,食道造影见食道静脉曲张明显减轻.本组无肺栓塞及死亡病例.结论 腔内治疗肝静脉阻塞型BCS方法简便、微创、有效,远期疗效尚有待于进一步观察研究.