Treatment of Budd-Chiari syndrome by transjugular intrahepatic portosystemic shunt

Patients with Budd-Chiari syndrome may require therapy with portal decompressive surgery. Transjugular intrahepatic portosystemic shunt has recently emerged as a new treatment alternative to surgical shunting, but reported literature is scarce and its long-term outcome is to be evaluated. The aim of our study was to present two patients with Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt. Two female patients with acute and subacute Budd-Chiari syndrome respectively, uncontrolled by medical therapy, were referred for transjugular intrahepatic portosystemic shunting. Both patients had refractory ascites, while one had hepatic insufficiency. Hepatic vein thrombosis was without definitive etiology in one patient and due to essential thrombocytosis in the other. Transjugular intrahepatic portosystemic shunt was successfully created in both patients; in one a transcaval approach was performed. Bare stents were used in one, while in the other a polytetrafluoroethylene-covered stent-graft. Technical success was 100% with no complications. Ascites resolved in both patients and liver function improved. The first patient remains asymptomatic with good shunt patency 6 months after intervention. The second patient is symptom-free two years after the shunt creation, despite stent occlusion. She remains asymptomatic due to hepatic vein collateral development. In patients with acute or subacute Budd-Chiari syndrome uncontrolled by medical therapy, transjugular intrahepatic portosystemic shunt is highly effective with no complications and an associated mid-term clinical success.     

Early experience of Budd-Chiari syndrome treatment with transjugular intrahepatic portosystemic shunt

The Budd-Chiari syndrome is a heterogeneous group of disorders characterized by obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. We present two cases of Budd- Chiari syndrome with severe ascites associated with polycythemia vera in first case and protein C deficiency in the second, in both cases transjugular intrahepatic portosystemic shunt were placed, with excellent control of symptoms, no mortality were observed, and just one episode of pulmonary venous thrombosis was observed. To our knowledge this is the first time that transjugular intrahepatic portosystemic shunt are used and reported in Budd-Chiari syndrome in Mexico.     

Transjugular intrahepatic portosystemic shunt in combination with oral anticoagulant for Budd-Chiari syndrome.

The antiphospholipid antibody syndrome is characterized by arterial and venous thrombosis including hepatic veins. Although transjugular intrahepatic portosystemic shunt or liver transplantation have been considered for Budd-Chiari syndrome, treatment options for patients with complete obstruction of three hepatic veins including the junction with the inferior vena cava are limited. We describe a 27-year-old female, who suffered thrombotic obliteration of hepatic veins including the portion confluent with the inferior vena cava (Budd-Chiari syndrome) associated with marked ascites and liver dysfunction. Transjugular intrahepatic portosystemic shunt using a Wall-stent (10 mm in diameter) between inferior vena cava and intrahepatic portal vein was performed. Intrastent coagulation and recurrence of thrombosis were prevented by combination therapy with warfarin potassium and ticlopidine hydrochloride. These treatments induced loss of ascites and improvement of liver function, and she has been able to resume daily life. The portosystemic shunt described above in addition to combination therapy with warfarin potassium and ticlopidine hydrochloride appeared to be one of the options for treating Budd-Chiari syndrome associated with antiphospholipid antibody syndrome.     

Successful emergency treatment with a transjugular intrahepatic portosystemic shunt for life-threatening Budd-Chiari syndrome with portal thrombotic obstruction

We report successful treatment of acute severe Budd-Chiari syndrome with portal venous thrombosis. The prognosis of patients with this condition is poor, because the therapeutic options are limited. A 38-year-old woman with polycythemia vera was admitted in a critical condition, and Budd-Chiari syndrome complicated by portal venous thrombosis was diagnosed. Tissue plasminogen activator and urokinase were infused systemically and were partially effective. Transjugular intrahepatic portosystemic shunting to reduce the high portal venous pressure was performed successfully and, eventually, her general condition improved. Our experience indicates that emergency transjugular intrahepatic portosystemic shunting is an effective therapeutic modality for controlling portal hypertension in patients with severe Budd-Chiari syndrome with portal venous thrombosis.     

Acute Budd-Chiari syndrome with liver failure: the experience of a policy of initial interventional radiological treatment using transjugular intrahepatic portosystemic shunt

BACKGROUND AND AIMS: The optimal management of acute Budd-Chiari syndrome (BCS) with liver failure is controversial. Options include anticoagulation, portal systemic shunting or liver transplantation. In recent years, transjugular intrahepatic portosystemic shunts (TIPS) have been tried in place of shunt surgery. We report our experience with a policy of initial interventional radiological treatment (TIPS) in this patient group. METHODS: A consecutive, non-selected series of five patients with acute BCS with liver failure presenting to our liver unit over a 36-month period from October 2000 were treated with a policy of initial attempt at transjugular shunt. RESULTS: Stents were successfully inserted in four patients. In one patient the hepatic vein remnant could not be cannulated. One of the four stented patients had a hepatic venous web. Rapid recurrent portal and hepatic vein thrombosis occurred in one patient despite anticoagulation and trans-shunt thrombectomy. This patient and the patient with failed stenting underwent successful liver transplantation. One successfully stented patient died unexpectedly at home 2 weeks after TIPS insertion. Four of five patients are alive (80%) at a mean follow-up time of 30 months. CONCLUSION: Initial TIPS, with liver transplantation for treatment failures, may be a reasonable, cost-effective and surgery-sparing treatment for acute Budd-Chiari presenting with liver failure.     

Transjugular intrahepatic portosystemic shunt by direct transcaval approach in patients with acute and hyperacute Budd-Chiari syndrome

When Budd-Chiari syndrome (BCS) is due to occlusion of all three hepatic veins, the standard transjugular intrahepatic portosystemic shunt (TIPS) technique can be extremely laborious. A feasible alternative is to use the direct transcaval approach, by which a shunt can be created directly between the intrahepatic inferior vena cava and the portal vein. We describe two patients (one with acute BCS and one with hyperacute BCS) who were successfully managed with this modified technique. Both patients recovered; one of them underwent elective liver transplantation 15 months after the procedure, whereas the other still had good hepatic function and a patent stent 24 months after the procedure. We conclude that, in selected patients with acute and hyperacute BCS, placement of a TIPS by the direct transcaval approach is a rapid and effective emergency procedure, which can either be curative or function as a bridge for elective liver transplantation.     

Intra-hepatic haematoma complicating transjugular intra-hepatic portosystemic shunt for Budd-Chiari syndrome associated with anti-phospholipid antibodies, aplastic anaemia and chronic hepatitis C

Portal venous decompression with transjugular intra-hepatic portosystemic shunt (TIPS) is a new approach in the treatment of Budd-Chiari syndrome. We report on a 31-year-old female with Budd-Chiari syndrome due to anti-phospholipid antibodies with compression of the inferior vena cava treated with TIPS and stenting of the inferior vena cava. TIPS was complicated by massive intra-hepatic haematoma which was managed conservatively. Treatment options and pathogenic mechanisms of Budd-Chiari syndrome under the rare coincidence of aplastic anaemia and anti-phospholipid syndrome are discussed. TIPS may be considered for venous decompression in Budd-Chiari syndrome, but physicians should be aware of potential TIPS' complications in these patients.     

Budd-Chiari syndrome, portal vein and mesenteric vein thrombosis in a patient homozygous for factor V Leiden mutation treated by TIPS and thrombolysis

We present the first case of Budd-Chiari syndrome in association with portal and mesenteric vein thrombosis in a patient homozygous for the factor V Leiden mutation. She was treated by transjugular intrahepatic portosystemic stent (TIPS) placement followed by local thrombolytic therapy. Venous outflow from the liver was established and the thrombi in the portal and mesenteric veins were lysed completely. This therapeutic approach may be used for such patients with this severe thrombotic event, who generally have a poor prognosis.     

Akute Gef??komplikationen der Leber

Acute hepatic vascular complications are rare. Acute portal vein thrombosis (PVT) and the Budd-Chiari syndrome (BSC) are the leading causes. Coagulopathy and local factors are present in up to 80% of cases. Diagnosis is established by colour-coded Doppler sonography, contrast-enhanced computed tomography or magnetic resonance imaging. Patients with acute PVT present with abdominal pain and disturbed intestinal motility. In the absence of cirrhosis anticoagulation with heparin is established followed by oral anticoagulation. In severe cases, surgical thrombectomy or transjugular thrombolysis with stent shunt may be necessary. Acute or fulminant BCS may require emergency liver transplantation or a transjugular intrahepatic portosystemic stent shunt, if patients present with acute liver failure. Milder cases receive anticoagulation for thrombolysis of occluded hepatic veins. Sinusoidal obstruction syndrome (SOS) is diagnosed after total body irradiation or chemotherapy, the term SOS replacing the former veno-occlusive disease. The treatment of congenital vascular malformations, complications in the setting of OLTX as well as patients with hepatic involvement of hereditary hemorrhagic telangiectasia requires significant expertise in a multidisciplinary approach.     

Budd-Chiari syndrome with fatal outcome in a patient with polycythemia vera and antiphospholipid antibody syndrome

We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.     

Acute hepatic vascular complications

Acute hepatic vascular complications are rare. Acute portal vein thrombosis (PVT) and the Budd-Chiari syndrome (BSC) are the leading causes. Coagulopathy and local factors are present in up to 80% of cases. Diagnosis is established by colour-coded Doppler sonography, contrast-enhanced computed tomography or magnetic resonance imaging. Patients with acute PVT present with abdominal pain and disturbed intestinal motility. In the absence of cirrhosis anticoagulation with heparin is established followed by oral anticoagulation. In severe cases, surgical thrombectomy or transjugular thrombolysis with stent shunt may be necessary. Acute or fulminant BCS may require emergency liver transplantation or a transjugular intrahepatic portosystemic stent shunt, if patients present with acute liver failure. Milder cases receive anticoagulation for thrombolysis of occluded hepatic veins. Sinusoidal obstruction syndrome (SOS) is diagnosed after total body irradiation or chemotherapy, the term SOS replacing the former veno-occlusive disease. The treatment of congenital vascular malformations, complications in the setting of OLTX as well as patients with hepatic involvement of hereditary hemorrhagic telangiectasia requires significant expertise in a multidisciplinary approach.     

USE OF TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT AS A BRIDGE TO TRANSPLANTATION IN FULMINANT HEPATIC FAILURE DUE TO BUDD CHURI SYNDROME

We report a case of fulminant hepatic failure in a 55-yr-old man due to Budd-Chiari syndrome in the setting of polycythemia rubra vera. The patient presented with acute hepatic failure, which rapidly progressed to grade IV hepatic encephalopathy. Placement of a transjugular intrahepatic portosystemic shunt resulted in marked improvement of the encephalopathy and stabilized the liver failure. Suhsequently, he underwent successful nonemergent orthotopic liver transplantation. Transjugular intrahepatic portosystemic shunt placement is a safe, effective, therapeutic option to bridge patients with fulminant Budd-Chiari to liver transplantation.     

Using transjugular intrahepatic portosystemic shunts to control variceal bleeding before liver transplantation.

OBJECTIVE: To determine the safety and efficacy of transjugular intrahepatic portosystemic shunts (TIPS) in controlling bleeding from esophageal varices in patients awaiting liver transplantation. DESIGN: Prospective, uncontrolled trial. SETTING: University medical center with an active liver transplant program. PATIENTS: Thirteen patients referred for liver transplantation with either active variceal hemorrhage or recurrent variceal hemorrhage despite sclerotherapy; four patients had been previously treated with surgical portosystemic shunts. INTERVENTION: An intrahepatic portosystemic shunt created via a transjugular approach to the hepatic veins using expandable, flexible metallic stents. MEASUREMENTS: Portal pressures before and after the creation of the shunt, the direction of portal blood flow at differing diameters of the shunts, procedure-related complications, and outcome in terms of survival, liver transplantation, and recurrent variceal bleeding. MAIN RESULTS: The transjugular intrahepatic portosystemic shunt was placed successfully in 13 patients, and bleeding was controlled acutely in all 13. After the procedure, the mean portal pressure decreased from 34 +/- 8.9 cm H2O to 22.4 +/- 5.4 cm H2O (P less than 0.001). No complications were associated with the procedure; however, two patients died of causes unrelated to the procedure. Seven patients subsequently underwent liver transplantation and are doing well, and three patients are being managed conservatively. Bleeding recurred in one patient 102 days after the procedure secondary to shunt occlusion caused by neointimal proliferation. CONCLUSION: Placement of a transjugular intrahepatic portosystemic shunt is apparently safe and effective therapy for variceal hemorrhage in patients referred for liver transplantation.     

Laparoscopic Sugiura procedure for conditioning of the blood stream through TIPSS in cirrhotic patient. Initial experience

The Sugiura procedure or the proximal gastric devascularisation was formerly the method of choice for esophageal varicose treatment in some patients. The frequency of this operation decreased stenting after the introduction of the transjugular portosystemic shunt into clinical practice. However this method performed laparoscopically could be useful as a complementary procedure when the blood stream through the transjugular intrahepatic portosystemic stent shunt is low and an esophageal rebleeding occurs. A 40-year old patient with hepatic cirrhosis and Child stage "B" was admitted to our clinic due to recurrent esophageal varicose bleeding. He underwent a transjugular intrahepatic portosystemic stent shunt implantation 27 months before the admission and the transjugular intrahepatic portosystemic stent shunt became occluded 3 times since implantation and was repeatedly revised. After admission a color Doppler of the stent was performed. The blood stream was 15 cm/s. The laparoscopic Sugiura procedure was performed after conditioning of the general status of the patient. Five ports were introduced 5 cm above the umbilicus, under the xiphoid, the right and left hypochondrium as well as the left mesogastrium. The dilated veins between the gastric coronary vein and esophagus and the short gastric veins on the great curvature were interrupted by means of an ultrasonic scalpel. The hiatus esophagus was opened, the esophagus was prepared up to the first pulmonal vein and the dilated esophageal veins were occluded with stitch ligatures. The operation was completed with Toupet partial fundoplication. The patient was followed 6 months after the surgery. No rebleeding occurred in this time period. Immediately after surgery the blood flow increased up to 97 cm/s; at 3 and 6 months follow-up the intrahepatic shunt remained open with 82 and 80 cm/s blood flow respectively. Laparoscopic Sugiura procedure performed as a complementary surgery after transjugular intrahepatic portosystemic stent shunt implantation increased blood perfusion through the intrahepatal constructed shunt and prevented its occlusion. However this initial experience has to be confirmed with a larger number of patients.     

Transjugular intrahepatic portosystemic shunt (TIPS) for budd-chiari syndrome

Summary The transjugular intrahepatic portosystemic shunt (TIPS) is widely used in the treatment of variceal hemorrhage and portal hypertension associated with cirrhosis. Its potential as a therapy for Budd-Chiari syndrome is less well-known. We report a case of a 15-year-old girl with Budd-Chiari syndrome who was successfully treated with a TIPS, leading to resolution of her ascites and improvement in liver function.     

Transjugular intrahepatic portosystemic shunt in the management of Budd Chiari syndrome

Budd Chiari syndrome presents with a wide range of severity and duration of symptoms. Transjugular intrahepatic portosystemic shunt has been used to treat selected Budd Chiari syndrome patients for several years. The technique of transjugular intrahepatic portosystemic shunt may be more challenging than in cirrhosis because of hepatic vein occlusion. Covered transjugular intrahepatic portosystemic shunt stents have reduced the requirement for follow-up interventions. Transjugular intrahepatic portosystemic shunt has been a successful bridge to liver transplant for Budd Chiari syndrome but is the definitive treatment in many cases. Patient selection is important to determine who will benefit from transjugular intrahepatic portosystemic shunt or other treatments such as hepatic vein recanalization or liver transplant.     

Successful treatment of chronic Budd-Chiari syndrome with a transjugular intrahepatic portosystemic shunt

Budd-Chiari syndrome is characterized by obstruction of the hepatic venous outflow tract. Therapeutic options for chronic Budd-Chiari syndrome are limited. We report the case of a 28-year-old woman who presented with recurrence of chronic Budd-Chiari syndrome with total obstruction of all major hepatic veins. Due to worsening liver function over the course of 1 year, she had to be listed for liver transplantation. Because of therapy-refractory ascites, declining renal function and severe esophageal varices, a transjugular intrahepatic portosystemic shunt (TIPS) was placed, planned as a bridge to transplantation. Following TIPS, a marked recovery of liver function could be observed, accompanied by disappearance of ascites, esophageal varices, and normalization of kidney function. Therefore, the patient could be removed from the waiting list for liver transplantation. This case demonstrates for the first time that the use of TIPS in chronic Budd-Chiari syndrome may result in marked recovery of liver function.     

The transjugular intrahepatic portosystemic stent-shunt (TIPS) as rescue therapy for complete Budd-Chiari syndrome and portal vein thrombosis

We present a 40-year-old female patient with epigastric pain, ascites, and progressive liver failure, caused by Budd-Chiari syndrome (BCS) with thrombotic occlusion of the right and middle hepatic veins. As underlying diseases, essential thrombocythemia and resistance to activated protein C (APC) due to heterozygote factor V Leiden were found. Initial therapy with heparin caused thrombocytopenia (HIT) type II culminating in thrombosis of the last patent left hepatic vein and further deterioration of liver function. The decision against a surgical shunt and liver transplantation by our surgeons on the basis of the risks involved, prompted us to insert a transjugular intrahepatic portosystemic stent-shunt (TIPS). There was no measurable flow signal in the doppler sonography of the portal vein presumably due to thrombosis. A further evaluation with magnetic resonance tomography and angiography was impossible due to movement artefacts. TIPS initially served as a diagnostic tool allowing direct angiography-diagnosed thrombosis of the portal vein, the superior mesenteric and the splenic vein respectively. However, insertion of the TIPS shunt and subsequent fragmentation led to an effective hepatic decompression and full recanalisation of the portal vein. In the present case TIPS simultaneously allowed the diagnosis of portal vein thrombosis and served as rescue therapy of complicated Budd-Chiari syndrome. The potential development of HIT type II should be kept in mind when heparin is given, especially to patients with thrombophilia.     

Transcatheter local thrombolysis in patients with extensive TIPS thrombosis

Background

Transcatheter local thrombolytic therapy in patients with portosplanchnic venous thrombosis has been used in few cases.

Case reports

Here, we present our single-center experience with transcatheter thrombolytic therapy in three patients with extensive refractory portal and transjugular intrahepatic portosystemic shunt (TIPS) thrombosis. Thrombolytic therapy was successful for all three patients. Two patients developed minor procedure-related bleeding.

Conclusion

Local thrombolysis could be proposed in case of TIPS thrombosis for patients in whom the venous flow cannot be restored by using conventional anticoagulant therapy and stent mechanical revision.     

Direct intrahepatic portocaval shunt (DIPS) or transjugular transcaval intrahepatic portosystemic shunt (TTIPS) to treat complications of portal hypertension: Indications,technique, and outcomes beyond Budd-Chiari syndrome

Transjugular intrahepatic portosystemic shunt (TIPS) is nowadays the benchmark treatment of severe portal hypertension complications. However, besides usual contraindication to the procedure (namely recurrent hepatic encephalopathy, severe liver dysfunction, right heart failure and/or pulmonary hypertension), TIPS appears regularly unfeasible due to abnormal and/or distorted anatomy. In this situation, the only non-surgical approaches to treat severe portal hypertension consist in the creation of an intrahepatic portocaval shunt from percutaneous (direct intrahepatic portocaval shunt - DIPS) or transjugular route (transjugular transcaval intrahepatic portosystemic shunt – TTIPS). These procedures have been rapidly adopted in patients with Budd-Chiari syndrome but are only poorly reported in patients with cirrhosis and without BCS. Considering the broadening landscape of TIPS indication in patients with cirrhosis within the last ten years, we aimed to describe the techniques, safety and efficacy of DIPS and TTIPS procedures as an alternative to TIPS in case of unfavourable anatomy.