超声引导下的腔内疗法或联合门体分流术治疗布-加综合征36例

目的 探讨布—加综合征腔内治疗的效果。方法 超声引导下行下腔静脉扩张和内支架植入术治疗布—加综合征36例(3例行单纯球囊扩张，33例行下腔静脉内支架植入)，其中13例因肝静脉闭塞，在介入术后1周行分流术，包括脾肾分流5例、肠腔分流8例。结果 超声介入治疗后，有3例出现轻度心功能不全。分流术后有1例出现胰腺炎，1例术后第10天死于上消化道出血。随访1个月至8年，1例2年后支架远心端出现狭窄，1例1年后肝静脉血栓形成，1例支架术后3年合并肝癌，1例1年后合并丙肝死亡，2例不孕症1年后得子。所有患者术后下腔静脉通畅，支架无移位，分流血管无血栓形成，门静脉高压症状明显缓解。结论 超声介入腔内治疗布—加综合征，方法简便、安全，疗效较好。对肝静脉全部闭塞者需加行门体分流术。该法为治疗某些类型的布—加综合征提供了一种可行和有效的方法。     

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.     

节段性狭窄闭塞Budd-Chiari综合征的介入治疗

目的　研究Budd Chiari综合征 (BCS)下腔静脉节段性狭窄闭塞合并肝静脉阻塞及血栓形成的介入治疗。方法　本组 13例下腔静脉节段性狭窄闭塞长 2～ 5cm ,其中 8例肝静脉闭塞 ,5例下腔静脉血栓形成。下腔静脉开通术应用房间隔穿刺针。闭塞肝静脉开通术应用RUPS 10 0肝穿装置。用 0 5～ 1 0cm球囊扩张 ,下腔静脉开通后用 1 0～ 2 0cm球囊扩张后放入金属内支架。术后抗凝治疗 3个月。结果　 13例患者成功的进行了经皮穿刺球囊扩张术 (PTA)和血管内支架植入治疗。术后患者肝脾缩小 ,腹水吸收。随访 3～ 2 6个月未见复发及消化道出血。结论　下腔静脉闭塞有血栓形成 ,必须在下腔静脉开通前用药物溶栓治疗。下腔静脉开通后放内支架要避开副肝静脉开口。肝静脉开通成形术应放在BCS介入治疗的首要位置。     

经颈内静脉肝内门体分流术治疗肝静脉广泛闭塞型布加综合征

目的探讨经颈内静脉肝内门体分流术(TIPS)治疗肝静脉广泛闭塞型布加综合征的临床疗效。方法采用TIPS治疗11例广泛肝静脉闭塞型布加综合征患者,其中3例为急性,8例为亚急性或慢性。患者表现为食管静脉曲张破裂出血和顽固性腹水,采用超声多普勒、CT或MRI、上消化道钡餐、血管造影和肝活检明确诊断。TIPS将肝内分流道建于肝后下腔静脉与门静脉分支,支架直径为10 mm,随访时间(63±43)个月。结果所有患者均成功完成TIPS,肝门部门静脉分叉处出血1例,1周后出血控制再植内支撑;肝内分流道建立后门体压力梯度由(41．2±10．5)cm H2O(1 cm H2O=0．098 kPa)下降至(12．4±4．7)cm H2O,门静脉血流速度由(11．2±2．8)cm／s增加至(52．2±13．7)cm／s。患者出血控制,腹水渐消退,肝功能指标明显好转。住院期间因肝功能衰竭死亡1例。术后随访,2例分流道狭窄分别行分流道再扩张或再植内支撑,其余8例无相关并发症。结论TIPS是治疗肝静脉广泛闭塞型布加综合征的重要方法,具有良好的远期疗效。     

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.     

肠腔分流或联合下腔静脉内支架置入术治疗布—加氏综合征

目的 探讨肠腔分流术或联合下腔静脉内支架置入术治疗布－加氏综合征（BCS）的效果。方法 回顾性分析15例肠腔分流术或联合下腔静脉内支架置入术治疗BCS的临床资料。结果 4例行肠腔侧侧直接吻合,11例应用PTFE人造血管行肠腔分流;12例联合下腔静脉内支架置入术,门静脉压降低10－27cmH2O(0.98J-2.65kPa)。1例术后因肝昏迷肝脏功能衰竭死亡。14例存活患者肝脾肿大缩小,腹水消失,无并发症,随访5－26个月,均恢复正常生活。结论 肠腔分流或联合下腔静脉内支架置入术成功率高,创伤小,疗效满意,是治疗BCS的有效实用术式。     

联合术式治疗布－加综合征

目的：探讨一种对布－加综合征达到联合减压目的治疗方法。方法：下腔静脉介入治疗（球囊主张加支架植入术）加肠系膜上静脉下腔静脉C型分流术9例,经右心房手指破膜加支架植入术4例。结果：13例术后肝脾肿大缩小,腹水消失或减少。下腔静脉压力降低0.49-1.08kPa(5-11cmH2O)。门静脉压力降低0.69-2.35kPa(7-24cmH2O)。12例存活,无并发症。1例术后因多器官功能衰竭死亡.随访3个月－5年,12例患者情况好,无腹水出现,恢复正常工作和生活。结论：新的组合术式扩大了治疗范围,既解除了下腔静脉高压,又降低了门静脉高压,达到了联合减压的目的,手术简化,减少创伤及患者痛苦,治疗效果满意。     

布加综合征的手术治疗及预后分析

目的总结布加综合征手术治疗的基本术式及治疗效果。方法回顾性分析笔者所在医院1994年4月至2013年12月期间收治的1024例布加综合征患者的临床资料,总结布加综合征手术治疗的基本术式及治疗效果。结果 1024例患者中,行外科手术116例,行介入手术908例;行下腔静脉单纯球囊扩张术265例,行下腔静脉球囊扩张加支架置入术464例,行肝静脉开通术97例,行经右心房-股静脉联合破膜球囊扩张支架成形术52例,行肠-腔静脉分流术7例,行根治性矫正术20例,行下腔静脉-右心房人工血管转流术45例,行腔-肠-房联合转流术6例,行腔-肠-颈联合转流术9例,行经颈静脉肝内门-体分流术30例,行肠-房转流术29例。术后获访902例,随访时间为1d-19年,平均13年。严格、灵活地应用各种术式治疗布加综合征为患者带来了令人满意的效果。结论布加综合征由多种原因所引起,其治疗应当根据不同的病因或病理类型而选择不同的治疗方法;对布加综合征的诊断与分型有助于我们制定安全、合理及有效的治疗方案。     

腔内血管技术用于治疗布加综合征15例分析

目的：探讨布加综合征（BCS）的腔内血管外科治疗。方法：在15例BCS患者中,应用PTA使闭塞或严重狭窄的下腔静脉成功再通后,13例同时置入血管内支架,4例肝静脉完全闭塞者在介入再通下腔静脉后1周内行肠系膜上静脉－下腔静脉间人工血管“H”型分流术。结果：在腔内介入治疗后,本组仅有1例患者出现轻度心功能不全,分流患者术后无严重手术并发症,随访2－42个月,除1例支架远端有部分再狭窄外,所有血管支架均通畅,无移位,分流血管无血栓形成,门体静脉高压症状明显缓解,结论：血管腔内介入治疗是BCS首选治疗方法。     

布加综合征介入治疗后支架金属支断裂取出1例报道

<正>病例资料患者,女,45岁,因"布加综合征下腔静脉支架植入术后9年"入院。9年前因"布加综合征、肝后段下腔静脉重度狭窄"在外院行"下腔静脉支架置入术"。3个月前复查腹部CTV见肝后段腔静脉支架受压变形(图1);肝静脉显影不清;奇静脉、半奇静脉增粗;脾静脉-左肾静脉分流。查体:腹围75 cm,腹平坦,未见腹壁静脉怒张;肝下界达右肋缘下1 cm,脾下缘达左肋缘下1 cm,移动性浊音阴性。     

Primary Budd-Chiari syndrome: outcome of endovascular management for suprahepatic venous obstruction

OBJECTIVE: Primary Budd-Chiari syndrome (BCS) is a rare form of hepatic venous outflow obstruction at the suprahepatic inferior vena cava (IVC), the hepatic veins, or both. We assessed our 4-year experience in the management of BCS to evaluate the results of our methods of care. METHODS: We conducted a retrospective review of a nonrandomized clinical trial conducted in three teaching hospitals. Among 28 primary BCS patients, 9 remained in medical treatment only, and 19 who failed to respond to medical treatment received additional endovascular (n = 17) or surgical therapy (n = 2). Nine underwent IVC balloon angioplasty alone, 6 had angioplasty plus stents, and 2 had transjugular intrahepatic portosystemic shunts (TIPS) for hepatic vein lesions. One patient had a mesoatrial bypass; another had liver transplantation. Immediate response to the therapy was assessed with angiography and ultrasonography based on anatomic and/or hemodynamic correction or reduction of the lesion. Subsequent assessment of portal hypertension status was made with periodic clinical and laboratory evaluation (eg, ultrasonography, liver biopsy). RESULTS: Twenty-six patients had had IVC stenosis or occlusion by focal or segmental lesion. Two patients had hepatic vein outlet obstruction. There was no evidence of coagulopathy as the pathogenesis; all were related to membranous obstruction of the vena cava. Excellent immediate response to the endovascular therapy and subsequent relief of portal hypertension were achieved in 14 patients. Four patients had restenosis or progression of the residual lesion within 2 years; three responded to repeated stenting. Primary patency was 76.5%, and primary assisted patency was 94.1%. Two patients with TIPS and two with surgical therapy maintained excellent results. The medical treatment remained effective only in a limited group of 6 (21.4%) of the 28 patients. CONCLUSIONS: In BCS, both endovascular and surgical interventions provide excellent results and potentially halt liver parenchymal deterioration caused by portal hypertension. Liver transplantation remains the ultimate solution for advanced liver failure.     

节段闭塞性布加综合征的腔内治疗

目的 探讨节段闭塞性布加综合征的腔内治疗经验.方法 回顾性分析45例节段闭塞性布加综合征的临床资料.分别采用下腔静脉开通术、经皮经腔血管成形术(percutaneoustransluminal angiophsty,PTA)及支架植入术治疗.复杂的病例采用三维数字减影血管造影(three-dimensional digital subtraction angiography,3D-DSA)技术进行多角度评估下腔静脉病变,寻找腔内治疗最佳工作角度.结果 本组45例中,43例成功穿通并扩张.下腔静脉压由术前的(35.3±3.9)cm H2O降至术后的(9.5±2.0)cm H2O,两者差异有统计学意义(t=43.68,P<0.01).2例下腔静脉穿通失败,改行腔房人工血管转流术.1例行PTA时发生急性心包填塞.35例获得随访,随访率77.8%(35/45),随访时间3～46个月,平均28.6个月.除1例术后15个月支架内血栓形成而改行腔房人工血管转流术外,其他34例患者无支架移位及肝静脉阻塞.心包填塞病例经手术修补下腔静脉后治愈,随访6个月除肋间神经痛外无其他不适.本组无肺栓塞及死亡病例.结论 节段闭塞性布加综合征腔内治疗的近期、中期疗效较好.3D-DSA技术有助于布加综合征的腔内治疗.     

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.     

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。     

经皮球囊导管成形支架植入术治疗布-加综合征的临床观察

目的 探讨经皮下腔静脉球囊成形加支架植入术治疗布加综合征(BCS)的临床疗效。方法 120例患者均经彩色多普勒超声、下腔静脉及肝静脉造影等检查明确诊断，并进行经皮下腔静脉球囊成形(PTA)加支架植入术(EMS)治疗。所有病例随访6-108个月。结果 扩张前静脉狭窄段内径0-6mm(平均3mm)，肝静脉内径2mm。扩张并置放血管内支架后，腔静脉、肝静脉内径分别为18—20mm和8mm。扩张前下腔静脉至右房压力差为18-35cmH20(1．76-3．43Da)，平均20．3cmH20(1．989kPa)，扩张后压差消失，119例手术顺利，1例左肝静脉轻度损伤。术后96例主要症状及体征消失或基本消失，19例明显改善。120例(100％)获1-108(平均67．35)个月随访，其中门5例在随访期间血管内支架形态良好，支架内血流通畅；3例在术后8，11，16个月发生腔静脉再阻塞，经二次PTA EMS治愈；2例于术后2—3个月发生3支肝静脉再阻塞，转为外科手术。结论 经皮下腔静脉球囊成形、支架植入术是非长段阻塞型布—加综合征良好的治疗方法。     

人体原位肝脏移植术后胆道与血管并发症的放射介入治疗

目的 评估放射介入治疗在人体原位肝脏移植病人术后胆道与血管并发症中的应用价值。方法 回顾性分析自1999年1月至2001年4月我院连续实施的48例人体原位肝脏移植的临床资料，总结放射介入技术治疗术后胆道与血管并发症的实施体会。结果 术后经T管胆道造影术及核磁共振胆胰管成像（MRCP）证实，3例病人存在胆道狭窄，其中2例为肝门部胆管与肝总管狭窄，1例为吻合口处胆管狭窄，共实施经T管窦道选择性球囊扩张术6例次，胆道狭窄得以改善，肝功能好转。术后经彩色多普勒超声、核磁共振血管造影（MRA）及静脉造影证实，2例病人存在肝上下腔静脉狭窄与门静脉吻合口狭窄，分别实施肝上下腔静脉狭窄段球囊扩张术1例次，肝上下腔静脉内支架置入术2例次、门静脉内支架置入术1例次。放射介入治疗后，影像学检查显示血管狭窄基本消失，病人肝功能明显好转、腹水显著减少、尿量增加。5例中最长1例健康存活至今已超过19个月。结论 肝脏主后应常规联合应用胆道造影术和MRCP了解胆树全貌，彩色多普勒超声和MRA监测血管血流动力学，以尽早诊断胆道和血管并发症；及时果断采用正确的放射介入技术处理胆道与血管并发症可取得良好疗效。     

The utility of TIPS in the management of Budd-Chiari syndrome

BACKGROUND AND AIM: Budd-Chiari syndrome (BCS) is a rare condition associated with hepatic venous outflow obstruction classically treated with portosystemic shunts or liver transplantation. Recent reports indicate promising results with the use of transjugular intrahepatic portosystemic shunts (TIPS) in the treatment of these patients. PATIENTS AND METHODS: We reviewed a 10-year single-institution experience with TIPS in patients diagnosed with BCS. RESULTS: Eleven patients with BCS underwent TIPS procedures, 3 of whom carried a diagnosis of paroxysmal nocturnal hemoglobinuria, a relative contraindication for liver transplantation. One TIPS procedure was unsuccessful for technical reasons. No patient suffered mortality or major morbidity related to the TIPS procedure. The mean reduction of portal venous pressures was 43.7%, with a mean decrease of 73% in the pressure gradient. Of the 7 patients where long-term follow-up was available, 57% had shunts which remained patent but required several nonsurgical revisions for occlusion, with an average assisted patency of 37.5 months. CONCLUSIONS: TIPS is an effective modality in the treatment of patients with BCS, especially for those who are not candidates for liver transplantation. TIPS can be successfully used as a bridge to surgical portosystemic shunting, as well as liver transplantation, but may cause technical difficulties when performing transplantation.     

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.