Severe venoocclusive disease after liver transplantation treated with transjugular intrahepatic portosystemic shunt

Venoocclusive disease (VOD) is due to hepatic sinusoidal lining injury leading to portal hypertension; its incidence after liver transplantation is about 2%. When severe, it does not respond to medical therapy and has a high mortality; retransplantation is the only therapeutic option. However, there are no detailed data regarding the use of transjugular intrahepatic portosystemic shunt for VOD after liver transplantation. We describe two patients who developed severe VOD after liver transplantation, failed defibrotide therapy, and were treated by transjugular intrahepatic portosystemic shunt (TIPS). The portal hypertension resolved completely and one had full histological recovery. We believe that TIPS should be attempted as it may resolve progressive portal hypertension and the hepatic congestion, while allowing the clinician time for listing for further liver transplantation if the patient fails to respond.     

Transjugular intrahepatic portosystemic stent shunt: indications and long-term outcome

The transjugular intrahepatic portosystemic stent shunt has replaced surgical shunt procedures as the standard therapy for complications of portal hypertension such as refractory ascites and recurrent variceal bleeding. However, reinterventions due to TIPS stenosis are necessary in 25-50 %. Major complications are the manifestation or worsening of hepatic encephalopathy. Recent studies using PTFE covered stents have shown lower stenosis rates and a trend towards prolonged survival.     

Transjugular intrahepatic portosystemic shunt vs. small-diameter prosthetic H-graft portacaval shunt: Extended follow-up of an expanded randomized prospective trial

We report herein the results of extended follow-up of an expanded randomized clinical trial comparing transjugular intrahepatic portosystemic shunt (TIPS) to 8 mm prosthetic H-graft portacaval shunt as definitive treatment for variceal bleeding due to portal hypertension. Beginning in 1993, through this trial, both shunts were undertaken as definitive therapy, never as a “bridge to transplantation.” All patients had bleeding esophageal/gastric varices and failed or could not undergo sclerotherapy/banding. Patients were excluded from randomization if the portal vein was occluded or if survival was hopeless. Failure of shunting was defined as inability to shunt, irreversible shunt occlusion, major variceal rehemorrhage, hepatic transplantation, or death. Median follow-up after each shunt was 4 years; minimum follow-up was 1 year. Patients undergoing placement of either shunt were very similar in terms of age, sex, cause of cirrhosis, Child’s class, and circumstances of shunting. Both shunts provided partial portal decompression, although the portal vein-inferior vena cava pressure gradient was lower after H-graft portacaval shunt (P<0.01). TIPS could not be placed in two patients. Shunt stenosis/occlusion was more frequent after TIPS. After TIPS, 42 patients failed (64%), whereas after H-graft portacaval shunt 23 failed (35%) (P <0.01). Major variceal rehemorrhage, hepatic transplantation, and late death were significantly more frequent after TIPS (P <0.01). Both TIPS and H-graft portacaval shunt achieve partial portal decompression. TIPS requires more interventions and leads to more major rehemorrhage, irreversible occlusion, transplantation, and death. Despite vigilance in monitoring shunt patency, TIPS provides less optimal outcomes than H-graft portacaval shunt for patients with portal hypertension and variceal bleeding. Presented at the Forty-First Annual Meeting of The Society for Surgery of the Alimentary Tract, San Diego, Calif., May 21–24, 2000.     

可控弹簧圈限制TIPS分流道血流治疗难治性肝性脑病

目的 观察Interlock可控弹簧圈限制分流道血流对TIPS术后难治性肝性脑病的干预效果。方法 对5例TIPS术后难治性肝性脑病患者以Interlock可控弹簧圈限制分流道血流,观察治疗效果。结果 5例共用7枚可控弹簧圈,其中10 mm×25 cm 3枚,15 mm×25 cm 1枚,10 mm×40 cm 3枚。限流术后配合内科对症治疗,1例患者明显好转,未出现肝性脑病症状;2例限流后2个月内仍反复发生肝性脑病,予以再次弹簧圈限流后症状消失;2例限流术后半个月出现腹胀、腹腔积液等门静脉高压症状,选用8 mm×60 mm球囊扩张原支架分流道处弹簧圈,植入8 mm×60 mm镍钛合金裸支架,之后未再出现肝性脑病及门静脉高压症状。结论 以可控弹簧圈限制分流道血流治疗TIPS术后难治性肝性脑病（5例）安全可靠。     

Liver transplantation in patients with liver cirrhosis and esophageal bleeding

BACKGROUND: Uncontrolled hemorrhage from esophageal varices is one of the most devastating complications of portal hypertension in patients with advanced cirrhosis. METHODS: Drug therapy, endoscopic therapy, transjugular intrahepatic portosystemic shunt (TIPS), or surgical shunts are used with increasing success in the prevention and treatment of bleeding. However, all these treatment modalities have limitations because they do not treat the liver cirrhosis itself. On the other hand, treatment modalities for variceal bleeding may influence the ease of the feasibility of the transplantation procedure. This is particularly the case for surgical treatments like portosystemic shunts and devascularization operations. For this reason these procedures should be avoided if possible. When positioned correctly, a TIPS provides an elegant way of treating portal hypertension without influencing the course of liver transplantation. Liver transplantation offers a treatment that cures both the portal hypertension and the liver disease. However, the use of this method of treatment is limited by the organ availability and by the organ allocation algorithm, resulting in considerable waiting time. CONCLUSION: In conclusion, esophageal bleeding should be noticed as an early warning factor, leading the hepatologist to consider liver transplantation and early listing of the patient.     

Transjugular intrahepatic portosystemic shunt as a bridge to liver transplant: Current state and future directions

Liver transplantation is one of the mainstays of treatment for liver failure due to severe chronic liver disease. Bridging therapies, such as placement of a transjugular intrahepatic portosystemic shunt (TIPS), are frequently employed to control complications of portal hypertension such as ascites, hydrothorax, and variceal bleeding, and thereby reduce morbidity in patients awaiting transplant. There is no significant difference seen in either graft survival or patient survival between those receiving TIPS pre-transplant and those who do not, although those receiving TIPS placement on average have a longer waiting time on the transplant waitlist. Locoregional therapies, such as thermal ablation or chemoembolization, can be efficacious in patients with HCC and pre-existing TIPS; however there is a risk for increased adverse events in patients receiving these therapies who have TIPS compared to those who do not. In summary, TIPS is a safe, effective treatment that can be used to ameliorate the complications that are sequelae of portal hypertension. While it does not appear to improve survival post-transplant, TIPS placement pre-transplant may increase survival time to transplant, thus improving overall survival as well as quality of life.     

Transjugular Intrahepatic Portosystemic Shunt: Present Status, Comparison with Endoscopic Therapy and Shunt Surgery, and Future Prospectives

During the 13 years since its introduction into clinical practice, transjugular intrahepatic portosystemic shunt (TIPS) has become widely accepted worldwide as a percutaneous, interventional procedure for treating complications of portal hypertension. An experienced, skillful team, however, is necessary to ensure the high technical success of TIPS and to avoid its potential procedural complications. Presently, TIPS is used mainly for treatment of acute or recurrent hemorrhage from gastroesophageal varices refractory to endoscopic therapy. Randomized studies have shown that it is more effective than endoscopic treatment for preventing rebleeding; however, it is associated with a higher incidence of encephalopathy. Both treatments produce comparable survival rates. TIPS is also effective in the treatment of hepatogenic ascites and hydrothorax and hepatorenal syndrome. In comparison with surgical shunts, TIPS is a significantly less invasive procedure that can be done in poor surgical candidates with advanced cirrhosis. The high rate of shunt obstructions seen with TIPS mandates close surveillance and maintenance, rendering TIPS a multistage procedure. This is a major disadvantage of TIPS compared to surgery. Presently, both TIPS and surgical shunts have their place in the treatment of gastroesophageal variceal hemorrhage unresponsive to endoscopic therapy. TIPS is most suited for class B and C patients, particularly those who are candidates for liver transplantation. Surgical shunts should be considered for patients with well preserved liver function. Large, randomized controlled studies should be done to compare these treatment methods. Animal experimental and early clinical studies using covered stents (stent-grafts) are promising for the prevention of shunt obstructions and thus converting TIPS from a multistage to a one-stage procedure.     

不同手术方式对门静脉高压症病人肝功能性血流量的影响

目的　探讨TIPS、断流术、断流加分流术对肝功能性血流量的影响。方法　本组肝硬化门静脉高压症病人 37例 ,行TIPS治疗 8例、断流术 10例、TIPS +门奇静脉断流术 10例、门奇断流+脾肾分流术 9例。采用超声多普勒、D 山梨醇 (SOD)清除率和直接门静脉测压检测手术前后肝总血流量、肝功能性血流量和门静脉压。结果　术前病人门静脉、肝动脉和肝总血流量显著增加 ,肝功能性血流量显著下降 ,ChildC级病人下降更为显著。TIPS、TIPS +断流术和断流 +脾肾分流术后门静脉压力和肝功能性血流量均明显下降 (P <0 .0 5 )。其中 ,TIPS术后肝功能性血流量下降显著大于TIPS +断流术和断流 +脾肾分流术。断流术病人门静脉压和肝功能性血流量无明显变化。结论　肝功能性血流是评估肝脏储备功能的重要指标 ,分流术在降低门静脉压力同时减少肝功能性血流量。     

Liver Transplantation Due to Iatrogenic Injuries: Two Case Reports

The transjugular intrahepatic portosystemic shunt (TIPS) is an acceptable procedure that has proven benefits in the treatment of patients who have complications from portal hypertension due to liver cirrhosis. In the literature few reports have described complications after TIPS placement. Initial surgery and local hemostasis have been needed to manage abdominal bleeding: if this treatment is insufficient, it may be necessary to perform a liver transplantation. This report describes the role of liver transplantation to manage dangerous complications in 2 patients after TIPS placement, when surgical procedures and hemostasis were unable to stop the bleeding.     

肝移植时代的门静脉高压治疗

胃食管静脉曲张出血是门静脉高压的常见并发症。药物和内窥镜治疗是静脉曲张的基础治疗。经颈静脉肝内门体静脉分流被推荐用于处理难治性或复发性胃食管静脉曲张出血。当患者存在危及生命的出血风险,而传统治疗风险较高、存在禁忌或效果不理想时,应选择肝移植治疗。传统治疗可以获得短期疗效,甚至可以较长时间稳定病情,但如果这些治疗导致门静...     

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??Surgical therapy procedure in cirrhosis with portal hypertension WU Zhi-yong, CHEN Wei. Department of General Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
Corresponding author??WU Zhi-yong, E-mail??zhiyongwu@gmail.com
Abstract Bleeding from esophagogastric varices is the most life-threatening complication of portal hypertension, which is the main target of traditional surgical therapy. It has been reached a consensus that non-operative therapy is primary during the period of acute variceal bleeding, such as pharmacotherapy, endoscopic therapy, triplelumen tube balloon tamponade and so on. In the case of refractory bleeding, emergency operation is suitable in patients provided that the liver dysfunction is not too severe ( Child- Pugh class A or B ). Devascularization is the most suitable choice in emergency operation so long as there is hepatopetal blood flow in the portal vein. Transjugular intrahepatic portosystem shunt (TIPS) is suitable for the patients of Child-Pugh class C who are in emergency state. Most patients who survive a first variceal hemorrhage episode should receive surgical treatment to prevent recurrent episodes. The etiological factor(s) should be defined before operation, and it also should be evaluated that the hepatic functional reserve, degree of portal hypertension and hemodynamics of the liver and portal system. Mainly for the traditional surgical method includes devascularization, shunt surgery and shunt combined with devascularization surgery. We emphasize that selection of operative method must be based on portal vein hemodynamics, and the operative modality must have a definite hemodynamic status. Among those who bleed in portal hypertension, patients with only liver function Child-Pugh class C who can not be improved by medical treatment (end-stage liver disease) are suitable for liver transplantation.     

采用Viatorr支架行TIPS治疗门静脉高压症疗效

目的分析Viatorr支架用于TIPS治疗门静脉高压的临床疗效。方法收集使用Viatorr支架行TIPS治疗的34例门静脉高压患者,分析术后门静脉压力下降情况、肝性脑病发病率及分流道通畅率。结果采用Viatorr支架行TIPS技术成功率100%;术前、术后门静脉压力分别为(40.00±3.85)cmH_2O和(23.60±2.87)cmH_2O。术后随访1～14个月,分流道通畅率100%(34/34),肝性脑病发病率5.88%(2/34)。结论使用Viatorr支架行TIPS治疗门静脉高压手术操作成功率高,分流道通畅率高,术后肝性脑病发生率低。     

TIPS序贯TACE、靶向和/或免疫治疗BCLC D期原发性肝细胞癌伴严重门静脉高压并发症

目的 观察TIPS序贯TACE、靶向和/或免疫治疗巴塞罗那临床肝癌（BCLC）D期原发性肝细胞癌（HCC）伴严重门静脉高压并发症的效果。方法 回顾性分析20例BCLC D期HCC伴严重门静脉高压并发症患者，均首先接受TIPS，之后序贯接受TACE、靶向和/或免疫治疗，观察治疗效果、并发症和预后。结果 20例TIPS均获成功，18例术中以弹簧圈或联合组织胶栓塞曲张静脉。TIPS前门体压力梯度为（32.25±4.51）mmHg，术后（18.05±7.54）mmHg，较术前降低（P<0.01），门静脉高压症状均得到有效缓解；未见手术相关并发症及围手术期死亡。术后1个月功能状态（PS）评分较术前降低（P<0.01）；17例肿瘤分期下降，其中14例降至BCLC C期、2例降至BCLC B期、1例降至BCLC A期。后续11例接受靶向治疗，接受TACE联合靶向治疗、TACE联合靶向及免疫治疗、靶向联合免疫治疗各3例。随访0~26个月，期间均未出现消化道出血，难治性腹腔积液均明显改善；未出现肝性脑病或分流道再狭窄。Kaplan-Meier生存曲线显示，术后3、6、12和24个月累积生存率分别为73%、61%、35%和4%。结论 TIPS序贯TACE、靶向和/或免疫治疗BCLC D期原发性HCC伴严重门静脉高压并发症安全、有效。     

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??Role of interventional radiology in the management of portal hypertension??current status WU Xing-jiang , LI Jie-shou. Department of General Surgery, General Hospital of Nanjiang Unit, People’s Liberation Army, Nanjing 210002,China Corresponding author:WU Xing-jiang, E-mail: wxj_wxj@sohu.com Abstract The interventional radiology plays a well-established role in the management of patients with complications of portal hypertension such as variceal bleeding or refractory ascites. Transjugular intrahepatic portosystemic shunt (TIPS) is an interventional radiology technique that has shown a 90% success rate to decompress the portal circulation?? TIPS has been in use for more than 20 years to treat the complications of portal hypertension. TIPS has been used in thousands of patients with liver disease worldwide. Portosystemic encephalopathy and stent dysfunction are TIPS major drawbacks. The improved results achieved with covered-stents might expand the currently accepted recommendations for TIPS use. Balloon-occluded retrograde transvenous obliteration (B-RTO) is another interventional radiology technique that can prevent and treat gastric variceal bleeding. The procedure has been widely performed in Japan.The bleeding control rate of gastric varices after B-RTO has been described as greater than 90%.     

介入放射技术治疗门静脉高压症进展

介入放射技术在治疗门静脉高压症食管静脉曲张出血和顽固性腹水中有着极其重要的作用。经颈内静脉肝内门体分流术（TIPS）用于治疗门静脉高压症已有20年历史,治疗数千例病人,技术成功率达90%,在全球广泛应用。TIPS虽存在肝性脑病和分流道阻塞的问题,近年来覆膜支架的应用显著提高分流道的通畅率。球囊阻塞的逆行胃底曲张静脉栓塞（B-RTO）是治疗胃底静脉曲张出血的另一种介入放射技术,在日本广泛应用,控制胃底静脉曲张出血成功率>90%。     

Biliary-Venous Fistula Complicating Transjugular Intrahepatic Portosystemic Shunt Presenting With Recurrent Bacteremia, Jaundice, Anemia and Fever

A 50-year-old White man with noncirrhotic portal hypertension presented with bleeding from gastric varices. Bleeding was initially managed with band ligation and subsequent transjugular intrahepatic portosystemic shunt (TIPS). Over the next few months, the patient had recurrent episodes of anemia, jaundice, fever and polymicrobial bacteremia. Computed tomography (CT) of the abdomen and chest, upper and lower endoscopy, endoscopic retrograde cholangiopancreatography (ERCP), and echocardiography failed to explain the bacteremia and anemia. Follow-up CT scan and Doppler sonography 9 months after placement showed TIPS was occluded. Repeat ERCP showed a bile leak with free run-off of contrast from the left hepatic duct into a vascular structure. The patient's status was upgraded for liver transplantation with Regional Review Board agreement and subsequently received a liver transplant. Gross examination of the native liver demonstrated a fistula between the left bile duct and the middle hepatic vein. Pathologic evaluation confirmed focal necrosis of the left hepatic duct communicating with an occluded TIPS and nodular regenerative hyperplasia consistent with noncirrhotic portal hypertension. Infection is rarely reported in a totally occluded TIPS. Biliary fistulas in patent TIPS have been treated by endoluminal stent graft and endoscopic sphincterotomy with biliary stent placement. Liver transplantation may be the preferred treatment if TIPS becomes infected following its complete occlusion.     

采用Viatorr覆膜支架行经颈静脉肝内门体分流术

目的评价采用Viatorr覆膜支架行经颈静脉肝内门体分流术(TIPS)治疗门静脉高压并发症的疗效与安全性。方法回顾性分析8例接受Viatorr覆膜支架TIPS治疗的肝硬化门静脉高压症患者的资料。术后进行随访,复查上腹部CT,以评价TIPS疗效。结果对8例患者均成功手术,均采用直径8mm、覆膜段长度50~80mm的Viatorr覆膜支架建立肝内分流道。对其中1例合并门静脉海绵样变的患者于门静脉端置入8mm×40mm的E-Luminexx裸支架1枚;1例患者因肝静脉端狭窄于肝静脉端置入8mm×40mm的Fluency覆膜支架1枚。术后患者门静脉压力由术前的[33.08(29.32,40.22)]mmHg降为[23.31(21.43,26.51)]mmHg,差异有统计学意义(Z=-2.52,P=0.012)。术后随访1.1~7.7个月,所有患者均存活,均未再发生门静脉高压相关并发症。术后2例患者发生肝性脑病。术后1~7.7个月复查示所有患者TIPS分流道通畅。结论对国内肝硬化门静脉高压症患者应用Viatorr支架行TIPS治疗安全、有效。     

Mesocaval shunt as an alternative treatment for persistent ascites after liver transplantation: case reports

Refractory ascites after liver transplantation is a relatively rare complication. If the initial medical treatment fails, more invasive techniques may be required. The TIPS procedure has emerged as a major treatment option for decompression of the portal venous system. Mesocaval shunt can be an alternative to TIPS in selected cases. We describe two patients who underwent mesocaval shunt construction for refractory ascites.     

Portal hypertension and ascites

Portal pressure is the product of portal blood flow and resistance; an increase in either leads to increased portal pressure. Cirrhosis is the underlying cause in most cases, but portal hypertension can develop due to pre-, intra- and post-hepatic obstruction to the flow, secondary to a variety of causes. Diagnosis can be established by a combination of non-invasive imaging or portal vasculature and clinical or serological markers for the cause underlying cirrhosis. Development of gastro-oesophageal varices and ascites are the most important clinical manifestation of portal hypertension. Non-selective β-blockers and endoscopic band ligation are effective in primary and secondary prevention of variceal bleeding. Active variceal haemorrhage is managed using a combination of vasoactive drug (e.g. terlipressin) and endoscopic band ligation. If these measures fail, transjugular intrahepatic portosystemic shunt (TIPS) insertion achieves haemostasis. Diuretic therapy with spironolactone and furosemide are the mainstays of management of ascites. If ascites becomes refractory, repeat large volume paracentesis and TIPS in selected cases help to control symptoms. Development of ascites is an important landmark in the natural history of cirrhosis and liver transplantation should be considered definitive treatment.     

The changing spectrum of treatment for variceal bleeding.

OBJECTIVE: The objective of this study was to assess the impact of endoscopic therapy, liver transplantation, and transjugular intrahepatic portosystemic shunt (TIPS) on patient selection and outcome of surgical treatment for this complication of portal hypertension, as reflected in a single surgeon's 18-year experience with operations for variceal hemorrhage. SUMMARY BACKGROUND DATA: Definitive treatment of patients who bleed from portal hypertension has been progressively altered during the past 2 decades during which endoscopic therapy, liver transplantation, and TIPS have successively become available as alternative treatment options to operative portosystemic shunts and devascularization procedures. METHODS: Two hundred sixty-three consecutive patients who were surgically treated for portal hypertensive bleeding between 1978 and 1996 were reviewed retrospectively. Four Eras separated by the dates when endoscopic therapy (January 1981), liver transplantation (July 1985), and TIPS (January 1993) became available in our institution were analyzed. Throughout all four Eras, a selective operative approach, using the distal splenorenal shunt (DSRS), nonselective shunts, and esophagogastric devascularization, was taken. The most common indications for nonselective shunts and esophagogastric devascularization were medically intractable ascites and splanchnic venous thrombosis, respectively. Most other patients received a DSRS. RESULTS: The risk status (Child's class) of patients undergoing surgery progressively improved (p = 0.001) throughout the 4 Eras, whereas the need for emergency surgery declined (p = 0.002). The percentage of nonselective shunts performed decreased because better options to manage acute bleeding episodes (sclerotherapy, TIPS) and advanced liver disease complicated by ascites (liver transplantation, TIPS) became available (p = 0.009). In all Eras, the operative mortality rate was directly related to Child's class (A, 2.7%; B, 7.5%; and C, 26.1 %) (p = 0.001). As more good-risk patients underwent operations for variceal bleeding, the incidence of postoperative encephalopathy decreased (p = 0.015), and long-term survival improved (p = 0.012), especially since liver transplantation became available to salvage patients who developed hepatic failure after a prior surgical procedure. There were no differences between Eras with respect to rebleeding or shunt occlusion. Distal splenorenal shunts (p = 0.004) and nonselective shunts (p = 0.001) were more protective against rebleeding than was esophagogastric devascularization. CONCLUSIONS: The sequential introduction of endoscopic therapy, liver transplantation, and TIPS has resulted in better selection and improved results with respect to quality and length of survival for patients treated surgically for variceal bleeding. Despite these innovations, portosystemic shunts and esophagogastric devascularization remain important and effective options for selected patients with bleeding secondary to portal hypertension.