64排螺旋CT对Budd-Chiari的诊断价值

目的探讨多层螺旋CT(multislice spiral CT,MSCT)血管成像技术在布-加综合征(Budd-Chiari syndrome,BCS)中的应用价值。方法使用64排螺旋CT机对19例经DSA或手术证实的布-加综合征于患者进行平扫及增强扫描,并采用图像后处理重建技术,显示肝静脉、下腔静脉肝内段血管的形态结构。结果 CTA清晰显示下腔静脉肝静脉阻塞的部位及侧支循环血管。根据梗阻部位分为:下腔静脉膜性阻塞型3例,节段性阻塞2例,肝静脉开口处阻塞型2例,肝静脉广泛性阻塞例3例,混合性阻塞型9例。结论 MSCT能准确直观反映布-加综合征阻塞的性质、部位、范围、侧支血管及其与周围器官的空间关系,对介入或手术治疗具有重要指导意义。     

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。     

Budd-Chiari syndrome resulting from a membranous web of the inferior vena cava: operative repair using profound hypothermia and circulatory arrest

A 47-year-old man was seen with Budd-Chiari syndrome caused by a congenital membranous web obstructing the inferior vena cava (IVC) above the hepatic veins. Operative repair was accomplished using cardiopulmonary bypass, profound hypothermia (24 degrees C), and 10 minutes of circulatory arrest. This technique permitted accurate resection of the web, thorough removal of thrombus from the IVC and the hepatic veins, and repair of the atriocaval junction with an autologous pericardial patch. Postoperative angiographic study showed unobstructed flow through the IVC with filling of the hepatic veins. We believe this is the first report of use of this technique to treat Budd-Chiari syndrome resulting from a congenital web obstructing the IVC and hepatic veins.     

Treatment of inferior vena cava obstruction producing Budd-Chiari syndrome

We treated a patient who had an inferior vena cava (IVC) obstruction associated with Budd-Chiari syndrome. All of the right, middle, and left hepatic veins were completely obstructed. The IVC was obstructed by a membranous substance and thrombus at the hepatic portion and was completely occluded by a fibrous septum at the site of a suprahepatic coarctation. A cavotomy was performed transversely at the suprahepatic level and then longitudinally to the level just above the renal veins, and the obstructing tissue was removed. An additional vertical incision was made in the IVC over the coarctation, and an autologous pericardial patch was sutured in place to widen the IVC. The patient was discharged with the patency of the IVC restored.     

Budd－Chiari综合征直视根治术

自１９８６年１月至１９９３年４月作者在常温血管阻断下经右胸腔切开下腔静脉直视根治Ｂｕｄｄ－Ｃｈｉａｒｉ综合征６７例，其中膜切除３７例次、血栓或癌栓摘除１９例次、心包片腔静脉扩大成形２８例次、心包片成管代血管移植３例和解除外压３例。手术死亡３例，２例死于出血性休克，１例死于右心衰。其他６４例治愈出院。术后随访５～８４个月者６４例，６１例典型表现均消失。Ｂ超和下腔静脉造影证实肝静脉和下腔静脉开放通畅者６１例，显示下腔静脉狭窄２例和下腔静脉血栓形成１例，取得了良好疗效。     

下腔静脉隔膜阻塞型Budd-Chiari综合征血流数值模拟

目的:应用计算机仿真的方法研究Budd-Chiari综合征(BCS)下腔静脉隔膜生长过程中血管的血流动力学变化。方法:基于MRA图像建立下腔静脉隔膜阻塞型BCS血管模型,利用Ansys Fluent软件进行血流参数仿真。结果:成功建立下腔静脉隔膜阻塞型BCS血管模型。模型实验结果显示,在隔膜生长过程中,在下腔静脉及3支主干肝静脉汇合处与隔膜之间有一低速区,该区域面积随隔膜的增长而逐渐变大;随着隔膜的发展,下腔静脉及肝右静脉低剪切力的区域不断扩大,而切应力变大。结论:下腔静脉隔膜的发生发展是一个缓慢变化的过程,可能与下腔静脉壁面剪切力的变化密切相关。     

A case of surgical correction of the inferior vena cava obstruction with Budd-Chiari syndrome

A 37-year-old man is presented with Budd-Chiari syndrome associated with a membranous obstruction of the inferior vena cava. Clinical symptoms were ascites, leg edema and dilatation of the superficial abdominal veins. Aggressive surgery was carried out for this case. Midsternal incision and right subcostal oblique incision were made, and hepatic segment of the inferior vena cava was reconstructed using pericardial patch after endovenectomy and partial cardiopulmonary bypass was used without temporary interruption of liver circulation. Blood flow from the right hepatic vein was restored. Postoperative course was not eventful and this patient remains free of any evidence of ascites and liver dysfunction after operation. This procedure may be useful particularly for protecting the liver function which is damaged by hemostasis.     

A radical operation for the Budd-Chiari syndrome associated with obstruction of the inferior vena cava. A report of six patients

The Budd-Chiari syndrome is caused by an occlusion of the hepatic veins and is often associated with an obstruction in the hepatic portion of the inferior vena cava (IVC). Therefore, the various shunt operations done in an attempt to relieve the portal hypertension are often not effective. By using a radical surgical technique on six patients with Budd-Chiari syndrome, the liver was freed and a wide longitudinal incision was made in the hepatic portion of the IVC. The obstructed hepatic vein was reopened using a Fogarty catheter, and a pericardial patch reinforced with a Teflon prosthesis was secured over the incision. Two patients were still asymptomatic 3 and 4 years after operation. Two patients died postoperatively of pneumonia and hepatic insufficiency. The two other patients were discharged in fair condition, and one died 6 months postoperatively following hepatic failure.     

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.     

Budd-chiari综合征血管造影诊断及分型

目的 通过造影表现,探讨Budd-chiari综合征的诊断及造影分型.方法 分别经下腔静脉双向插管造影、经下腔静脉肝静脉造影及经皮肝穿肝静脉造影对患者进行造影诊断.结果 在所有342例经造影证实的Budd-chiari综合征病例中,下腔静脉病变207例,占60.5%,其中下腔静脉膜性病变142例,占41.5%,阶段性病...     

Therapeutic experiences with Budd-Chiari syndrome: report of 100 cases

One-hundred cases of Budd-Chiari syndrome were treated from December 1982 to March 1988. There were 62 males and 38 females. Their ages ranged from 15 to 62 years averaged 32.6 years Seventy-six cases had intractable ascites, 56 had esophageal varices and 22 had upper gastrointestinal bleeding. There were 37 cases with membranous obstruction, 57 with occlusion of the inferior vena cava above the confluence of the hepatic veins and 6 with occlusion of hepatic veins. Eighty-one cases (81%) were operated upon. Operative mortality was 8.6% (7/81). Follow-up from 2 to 63 months revealed that 60 operated cases (74.1%) had good results, while 11 of 19 (58%) patients treated conservatively died within two months after admission. It is concluded that the operative procedure must be tailored to the etiology and pathology. The mesoatrial shunt is the operation of choice for occlusion of the retrohepatic IVC and hepatic veins, the cava-atrial shunt for occlusion of the IVC and patent hepatic veins, membranotomy for IVC web, and the meso-caval shunt for intrahepatic venous occlusion. For those who also have superior vena cava compression syndrome, an innominate-atrial shunt should be added.     

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.

     

Membranous obliteration of the inferior vena cava in the hepatic portion--postmortem study of a rapidly deteriorated aged case

A 67-year-old female of Budd-Chiari syndrome was rapidly deteriorated and died. Autopsy revealed membranous obliteration of the inferior vena cava in the hepatic portion with a small hole. The inferior vena cava and right hepatic vein contained fresh and organized thrombi. The left hepatic vein was patent and opened above the membrane. The onset and the clinical course of this case seem to be atypical, probably because of the patent left hepatic vein and a small hole of the membrane. We call attention such a rare case in managing Budd-Chiari syndrome.     

Liver transplantation in patients with Budd-Chiari syndrome

Patients with Budd-Chiari syndrome (obstruction of the hepatic veins) and associated hepatic insufficiency may be candidates for orthotopic liver transplantation (OLT). In our series of 405 OLT patients, 3 were transplanted due to Budd-Chiari syndrome (0.7%). The indication for liver transplantation in these patients was severe hepatic insufficiency (chronic in two and acute in the third one). Morphologic study of the obstructions revealed apparently different causes, including thrombi, membranous webs in hepatic veins, and hydatid cyst compression. The surgical technique employed in these transplantations was similar to that for other etiologies. Due to its implications for the future course of OLT, it is important to determine the exact etiology of Budd-Chiari syndrome in the pretransplant period and to treat the patients with early and long-term anticoagulant therapy to avoid syndrome recurrence.     

Living donor liver transplantation for Budd–Chiari syndrome with hepatic inferior vena cava obstruction after open pericardial procedures

Living donor liver transplantation (LDLT) for Budd–Chiari syndrome (BCS) presents a unique challenge as it does not involve replacement of the hepatic inferior vena cava (IVC). We report a case of successful LDLT in a patient with BCS associated with occlusion of the hepatic veins as well as the IVC. A 34-year-old woman with a history of two open pericardial procedures had decompensated liver failure and portal hypertension. Venography showed complete obstruction of the hepatic IVC and well-developed collateral vessels. We performed LDLT via sternotomy and laparotomy, with an end-to-end anastomosis between the left hepatic vein of the donor and the patient’s suprahepatic vena cava in the pericardium. The patient recovered uneventfully and has been doing well for 5 years. LDLT without caval replacement for BCS in a patient with hepatic IVC obstruction is feasible if the patient has good functional collaterals before liver transplantation.     

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。     

Utilization of autologous vein graft for replacement of the inferior vena cava in living-donor liver transplantation for obliterative hepatocavopathy

Obliterative hepatocavopathy (OHC) is a subtype of Budd-Chiari syndrome in which stenosis or obstruction of the retrohepatic inferior vena cava (IVC) is observed. Although IVC replacement is necessary in OHC patients, there are hardly any graft vessels available for IVC reconstruction during living-donor liver transplantation (LDLT). Here, we describe a novel technique of IVC reconstruction using only the autologous blood vessels in an OHC patient during LDLT. In this case, sufficient drainage of the hepatic outflow and reconstruction of the venous return from the lower half of the body were simultaneously required. Therefore, we substituted the retrohepatic IVC with the suprarenal IVC of the recipient, and we reconstructed the IVC continuity by using the autologous internal jugular vein and external iliac vein. The operation was safe, and the postoperative venous drainage from the hepatic tributaries was in good condition. This procedure might be an option for IVC replacement during LDLT.     

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.     

布-加氏综合征的超声特征性变化

目的探讨布-加氏综合征(B-CS)二维及彩色多普勒超声特征。方法对21例临床疑诊B-CS的患者进行彩色多普勒超声检查并与X线血管造影对照。结果14例确诊为B-CS;肝静脉间可见多条交通支形成;肝静脉狭窄8例,闭塞1例;下腔静脉狭窄5例。肝静脉血流经交通支分流到未闭的肝静脉或经肝尾叶静脉及其他扩张的静脉分流入下腔静脉。结论肝静脉、下腔静脉狭窄及闭塞和肝静脉间交通支形成是B-CS超声特征性改变,结合血流动力学变化可以准确诊断B-CS。     

直视根治术治疗Budd—Chiari综合征的系列临床研究

目的 为进一步明确Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的病因、病变、分型、诊断和治疗方法。方法 对１９８６年１月～１９９５年７月采用要静脉切开直视根治术证实的７０例Ｂｕｄｄ－Ｃｈｉａｒｉ综合征进行系统临床分析。结果 病因依次为腹内感染、口服避孕药、血液凝固异常、腹外伤、肿瘤和索带嵌压等;病变均在下腔静脉,尚未见单纯肝静脉病变。所有病人均采用常温下腔静脉血流阻断或置管转流下,施行直视台术,疗效满意。结论 该