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.     

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。     

经腹肝上下腔与肝后下腔静脉人工血管架桥术治疗Budd-Chiari综合征

目的:探讨治疗Budd-chiari综合征有效的手术方式。方法:肝后下腔静脉短节段闭塞型Budd-chiari综合征,16例采用经腹行肝上下腔静脉与肝后下腔静脉人工血管架桥术(改良手术组),相同病理类型18例应用开胸行右心房与肝后下腔静脉人工血管架桥术(传统手术组),进行疗效对比研究。结果:改良手术组16例中,术中1例急性右心衰,15例术中经过顺利。人工血管长度6~8cm,随访6~55个月人工血管内无血栓形成。传统手术组18例中,术中急性心包填塞1例。术后发生胸腔积液3例,肺部感染与急性心包炎各1例。人工血管长度12~14cm。术后随访60个月,有3例分别于术后37、42、58个月出现人工血管内血栓。结论:经腹腔行肝上下腔静脉与肝后下腔静脉人工血管架桥术临床效果优于传统的开胸行下腔静脉右心房人工血管架桥术,该手术是治疗肝后下腔静脉短节段闭塞型Budd-chiari综合征安全有效的手术方式。     

下腔静脉型布加综合征合并下腔静脉血栓形成的治疗及随访分析

目的探讨下腔静脉（inferior vena cava,IVC）型布加综合征（Budd-Chiari syndrome,BCS）合并IVC血栓形成的治疗方法、疗效及预后。方法回顾性分析2004年4月至2012年6月期间因膜性或短段闭塞性IVC型BCS合并IVC血栓形成于郑州大学第一附属医院行介入治疗的128例患者的临床资料,比较行小球囊预开通治疗及可回收支架治疗患者的疗效。结果 128例患者中,9例行搅拌溶栓治疗（搅拌溶栓组）,56例行小球囊预开通治疗（预开通组）,63例行可回收支架治疗（可回收支架组）。除可回收支架组有1例患者于术中发生支架断裂、行外科手术外,其余127例患者的介入手术均成功。术后可回收支架组发生支架移位2例,余2组无并发症发生。预开通组与可回收支架组的尿激酶用量、溶栓时间、住院时间以及总并发症发生率比较差异均无统计学意义（P〉0.05）,但可回收支架组的住院费用高于预开通组（P〈0.01）。术后128例患者均获访,随访时间18-66个月,平均44.2个月。随访期间,搅拌溶栓组有1例、预开通组有6例、可回收支架组有6例患者发生原闭塞处的再次狭窄闭塞,预开通组与可回收支架组的再次狭窄闭塞率比较差异无统计学意义（P〉0.05）。复发患者经再次扩张治疗后未再发生管腔狭窄和血栓形成。结论搅拌溶栓技术治疗IVC型BCS合并IVC新鲜血栓形成安全而有效;小球囊预开通技术及可回收支架技术治疗IVC型BCS合并IVC陈旧性血栓形成的效果满意,且前者更经济。     

Budd-Chiari综合征介入治疗138例分析

目的探讨不同类型Budd-Chiari综合征(Budd-Chiari syndrome, BCS)介入治疗的选择和疗效. 方法 138例BCS根据下腔静脉(inferior vena cava,IVC)和肝静脉造影分为4种主要类型(I型,IVC膜性狭窄、闭塞;Ⅱ型,IVC节段性狭窄;Ⅲ型,IVC节段性闭塞;Ⅳ型,IVC基本通畅)和9种亚型.治疗方法:经股静脉、右颈静脉或锁骨下静脉进行下腔静脉球囊扩张成形术(percutaneous transluminal angioplasty,PTA)和支架放置术;经皮经肝、经右颈静脉或下腔静脉行肝静脉PTA和支架放置术. 结果 I型成功率100%(83/83),Ⅱ型成功率75.0%(9/12),Ⅲ型成功率81.6%(31/38),Ⅳ型成功率60.0%(3/5).死亡1例(0.7%,1/138).并发症4例(2.9%,4/138),其中误穿心包2例,支架脱落1例,肝脏出血1例.治疗成功的126例随访3～96个月,平均26个月.复发8例,其中2例死亡;肝癌死亡2例. 结论介入治疗BCS创伤小,操作简单,相对安全,疗效满意,成为首选的治疗方法.     

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。     

腔内治疗肝静脉阻塞型布加综合征

目的 探讨肝静脉型布加综合征(Buddi-Chiari syndrome,BCS)介入治疗方法和疗效的评估.方法 37例肝静脉型BCS患者,术前常规行腹部彩超检查,CT扫描及肝脏血管重建.采用经颈静脉、经股静脉或二者联合途径行肝静脉穿刺造影及闭塞段的开通.术后给予护肝、抗凝、溶栓治疗.结果 手术成功34例,成功率92％,共开通了38条肝静脉.肝静脉开通后,单纯行球囊扩张患者9例;余25例患者置入肝静脉支架27枚,其中2例患者同时置入了右肝静脉和副肝静脉的支架.闭塞的下腔静脉开通后置入国产Z型支架7枚.肝静脉的压力术前24 ～ 48 cm H2O,平均为(36.0±3.4)cm H2O,介入治疗后即刻下降为11～34 cmH2O,平均(21.0±2.3) cm H2O.术后随访3 ～48个月,平均(23.0±2.0)个月,9例单纯行球囊扩张术治疗的患者中,有4例再次发生狭窄或闭塞,均再次行支架置入治疗.行支架置入的患者23例得到了随访,其中6例发生再狭窄或闭塞(6/23,26％).结论 肝静脉型BCS可根据肝静脉闭塞和肝内侧支建立的情况行介入治疗,可显著降低肝静脉和门静脉压力,改善患者的临床症状.     

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.     

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。     

布一加综合征继发腹腔间隔室综合征35例

我科1990年以来,收治布—加综合征(Budd-Chiari syn-drome,BCS)继发腹腔间隔室综合征(abdominal compartmentsyndrome,ACS)35例,现报告如下.1 临床资料1.1 一般资料 35例中,男21例,女14例,年龄18～43岁,平均28.3岁.病程10天～3个月,平均43天.病变类型:单纯肝静脉(Ⅱ型)28例,长节段下腔静脉闭塞合并主肝静脉闭塞(Ⅲ型)7例.全部病例均经B超或Doppler超声检查结合下腔静脉造影和/或经皮肝穿刺肝静脉造影确立BCS诊断和分型.根据临床综合表现和腔内压测定确立ACS诊断.     

序贯介入治疗Budd-Chiari综合征合并下腔静脉新鲜血栓

目的观察序贯介入治疗Budd-Chiari综合征合并下腔静脉(IVC)新鲜血栓的有效性及安全性。方法对27例Budd-Chiari综合征合并IVC新鲜血栓患者行小球囊预扩张、置管溶栓及大球囊扩张等序贯介入治疗;记录不良反应;术后随访复查超声,评估治疗效果。结果对27例均以直径10～14 mm小球囊预扩张IVC闭塞段,扩张后原闭塞段血流均部分恢复;经留置溶栓导管泵入尿激酶40～60×10~4U/d、平均(44.10±8.40)×10~(4 )U/d,持续4～15天、平均(6.72±5.21)天后,超声显示20例IVC内血栓完全消失,7例仍残留少量陈旧血栓;以大球囊(直径25～30 mm)扩张IVC原狭窄段,之后IVC血流恢复,侧支血管明显减少或消失。围手术期未见肺栓塞、大出血等严重并发症。术后平均随访(20.22±18.43)个月,27例患者均存活,相关临床症状及体征消失或不同程度缓解。结论序贯介入治疗Budd-Chiari综合征合并IVC新鲜血栓安全、有效。     

下腔静脉及肝静脉短段病变型布-加氏综合征的外科治疗

目的 探讨布-加氏综合征的下腔静脉短段闭塞及肝静脉主干病变根治性切除的优缺点、术中的注意事项。方法 下腔静脉闭塞或狭窄段切除、人工血管原位移植术42例,肝静脉主干闭塞段切除、肝静脉流出道成形、人工血管原位移植术10例,肝静脉主干闭塞段切除、肝静脉-右心房吻合3例,经皮肝穿肝或副肝静脉破膜、扩张10例。结果 1例死于并发症,随访58例,平均30个月,介入复发3例,手术复发1例。结论 下腔静脉或肝静脉病变段切除、人工血管原位移植治疗布-加氏综合征,能根治病变、符合正常解剖生理、远期疗效好。     

Treatment of Budd-Chiari Syndrome by Side-to-Side Portacaval Shunt: Experimental and Clinical Results

The Budd-Chiari syndrome caused by occlusion of the major hepatic veins, often of unknown etiology, is typically characterized by massive ascites, hepatomegaly and abdominal pain due to intense congestion of the liver. The outcome has almost always been fatal. This report describes an evaluation of side-to-side portacaval shunt in dogs with experimental Budd-Chiari syndrome and in six patients with hepatic vein thrombosis. In the animal studies, side-to-side portacaval shunt was very effective in relieving massive ascites, hepatomegaly, hepatic congestion and portal hypertension produced by ligation of the hepatic veins. Only one of 24 dogs with side-to-side anastomosis reformed ascites, 67% of the animals survived until the study was concluded after one year, and liver biopsies showed reversal of the severe pathologic abnormalities. In contrast, all 20 control dogs subjected to a sham laparotomy, and all 20 dogs that underwent end-to-side portacaval shunt reformed massive ascites and died within six months with continued hepatic congestion and necrosis.

All six patients with the Budd-Chiari syndrome due to hepatic vein occlusion had massive ascites (4.4-15.9 l), hepatomegaly, abdominal pain and disturbed liver function. In all six, angiography demonstrated occlusion of the hepatic veins with a patent inferior vena cava (IVC) and a normal IVC pressure, and liver biopsy showed intense centrilobular congestion and necrosis. The most valuable diagnostic study was angiography of the IVC and hepatic veins with pressure measurements. Side-to-side portacaval shunt was performed from four to 14 weeks after the onset of symptoms, and produced dramatic and sustained relief of ascites in five of the six patients during follow-up periods of from eight months to seven years. Liver function returned to normal, hepatosplenomegaly disappeared, none of the survivors developed portal-systemic encephalopathy, and follow-up liver biopsies showed disappearance of congestion and necrosis, but mild to moderate fibrosis. One patient died following an emergency IVC thrombectomy and portacaval shunt, which was undertaken when, during the course of his workup, his condition deteriorated suddenly because the thrombotic process extended from the hepatic veins into the IVC. The everpresent risk of this complication, and the dangers associated with delaying operation were emphasized by this case. It is concluded that side-to-side portacaval shunt, which decompresses the liver by converting the portal vein into an outflow tract, provides effective treatment of the Budd-Chiari syndrome when the occlusive process is confined to the hepatic veins.

     

桥式背驮式肝移植治疗布加综合征:附六例次临床病例分析

目的探讨肝移植治疗布加综合征的外科新技术。方法1999年以来笔者共为6例布加综合征病人施行了桥式背驮式肝移植,通过血管架桥将供肝的肝上下腔静脉与受者的右心房吻合,供肝的肝下下腔静脉与受者的下腔静脉吻合。结果6例次肝移植术均获成功,未发生手术技术并发症。其中1例术后3个月死于多器官功能衰竭,其他5例仍在随访中,1例已存活近7年,术后均未出现布加综合征复发。结论桥式背驮式肝移植为临床治疗布加综合征提供了一种新的技术途径,减少了技术并发症和布加综合征复发可能。     

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.     

Budd-Chiari syndrome treated by Senning's procedure

Two patients with Budd-Chiari syndrome who underwent a new surgical procedure developed by Senning are reported. A 33-year-old man was diagnosed as having Budd-Chiari syndrome with a membranous obstruction of the inferior vena cava (IVC) and right hepatic vein and short segmental obstruction of the left hepatic vein. Removal of the obstruction by dorsocranial resection of the liver and reconstruction of the veins by hepatoatrial anastomosis was carried out. In a 53-year-old female, the same procedure was carried out for a short segmental obstruction of the IVC and left hepatic vein. In both patients, postoperative examination revealed good patency of the IVC and the hepatic veins and increased portal venous flow as measured by Doppler-echography. This procedure is considered the method of choice for Budd-Chiari syndrome with membranous and/or short segmental obstruction of the IVC and hepatic veins.     

CT指导介入治疗下腔静脉Budd-Chiari综合征

目的评估CT对下腔静脉型Budd-Chiari综合征(BCS)介入治疗的临床指导价值。方法收集于我院接受介入治疗的329例下腔静脉型BCS患者,所有患者术前均接受CT检查,并根据CT诊断结果制定介入治疗方案。以DSA治疗结果为金标准,评估CT诊断的准确率、敏感度、特异度、阳性预测值和阴性预测值。结果 DSA示下腔静脉不完全闭塞型BCS 108例,下腔静脉完全闭塞型221例;CT诊断下腔静脉不完全闭塞型99例,下腔静脉完全闭塞型230例。与DSA结果相比,CT诊断15例假阴性,6例假阳性。CT术前制定是否破膜的准确率、敏感度、特异度、阳性预测值及阴性预测值分别为94.19%、97.29%、86.11%、93.49%及93.94%,CT术前制定介入治疗方法的准确率、敏感度、特异度、阳性预测值及阴性预测值分别为97.55%、100%、92.52%、96.49%和100%。结论 CT诊断下腔静脉型BCS准确率较高,且有助于制定介入治疗方案。     

Budd-Chiari综合征的低场强MRI诊断价值

目的探讨低场强MRI对Budd-Chiari综合征的诊断价值。方法对12例经血管造影证实的Budd-Chiari综合征患者的MRI表现进行回顾性分析。结果肝脏体积增大伴尾叶增大9例，明确显示下腔静脉狭窄或闭塞7例，肝外侧枝血管形成7例，腹水8例。结论低场强MRI对Budd-Chiari综合征的诊断有重要的价值。     

Supradiaphragmatic approach for Budd-Chiari syndrome with transjugular intrahepatic portosystemic shunt stent in combination with inferior vena cava reconstruction during living donor liver transplantation: a case report

Treatment of Budd-Chiari syndrome consists of medical management, surgical shunt, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation. Liver transplantation is indicated only when other treatments have failed. A 36-year-old Japanese man underwent living-donor liver transplantation after radiologic intervention procedures. Because of the position of the TIPS stent and the damaged vascular lesion of Budd-Chiari syndrome, a supradiaphragmatic approach was employed to achieve a safe total hepatectomy. Moreover, after resection of damaged portion of the inferior vena cava (IVC), an artificial vascular graft was utilized to fill the IVC gap. The postoperative course was uneventful; no serious complications were experienced within 2 years after liver transplantation. This supradiaphragmatic IVC approach and IVC reconstruction technique emphasized the option of surgical techniques to decrease the operative risk during liver transplantation for Budd-Chiari syndrome.     

布-加综合征合并下腔静脉血栓介入治疗术后复发危险因素分析

目的 分析布-加综合征（B-CS）合并下腔静脉（IVC）血栓病人介入开通后的复发情况,并探讨其危险因素。方法 回顾性分析2015年10月至2020年10月在郑州大学第一附属医院接受介入治疗的79例B-CS合并IVC血栓病人临床资料并进行随访,使用Kaplan-Meier法计算术后复发率并绘制复发曲线,对影响复发的危险因素进行Cox回归分析。结果 42例病人于首次术后出现复发,6个月、1年、2年、3年、5年的累积复发率分别为24.3%、32.5%、51.0%、56.5%、69.7%。36例复发病人再次接受了介入治疗,其中14例IVC阻塞病人二次术后6个月、1年、2年的累积复发率分别为7.1%、15.6%、26.1%;另外22例合并血栓形成的病人二次术后累计复发率为14.3%、33.3%、38.9%。多因素Cox回归分析显示IVC阻塞段长度、A类副肝静脉数目、口服抗凝药类别、血小板计数、血清白蛋白、New Clichy指数是病人复发的独立危险因素（P值均＜0.01）。结论 B-CS合并IVC血栓的介入术后复发率较高并受多种因素影响,定期复查、尽早干预能够改善病人预后。