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.     

Budd‐Chiari and inferior caval vein syndromes due to membranous obstruction of the liver veins: successful treatment with angioplasty and transcaval transjugular intrahepatic porto‐systemic shunt

The case is presented of a 25‐year‐old Caucasian patient with Budd‐Chiari syndrome due to membranous obstruction of the liver veins and inferior caval vein syndrome as a result of secondary hyperplasia of the caudate lobe of the liver, obstructing the caval vein. Diagnosis was established by intravascular pressure measurements, ultrasound examinations and caval and liver vein angiograms. Treatment consisting of stent placement in the outlet of a hepatic vein and subsequent transjugular intrahepatic porto‐systemic shunt (TIPS) insertion via the caval vein was successful. After 34 months of follow‐up the stents remain open and the patient is symptom free. This successful combination of stent placement and TIPS has not been described before. The case report is followed by a review of the literature on the use of angioplasty in short hepatic vein stenosis and TIPS in Budd‐Chiari syndrome. It is concluded that angioplasty and TIPS are safe and efficient procedures to reduce liver engorgement and complications of portal hypertension in selected patients with Budd‐Chiari syndrome.     

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.     

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.     

Budd–Chiari syndrome: Focus on surgical treatment

Budd–Chiari syndrome (BCS) is caused by an obstruction in the hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava (IVC) due to thrombosis or fibrous sequelae. This rare disease mainly affects young adults. Risk factors have been identified and patients often have multiple risk factors. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients in Europe and North America countries. Multistep management is required for such patients. Interventional revascularization and transjugular intrahepatic portosystemic shunt procedure are indicated after initial anticoagulation therapy, whereas IVC plasty using a patch graft is indicated for obstruction of the IVC. Liver transplantation (LT) is usually indicated as a treatment for liver failure despite various treatments. The outcomes of LT are good, with a 5‐year survival after LT of nearly 70%.     

The use of TIPS in chronic liver disease

The development of cirrhosis and portal hypertension in the natural history of chronic liver disease is associated with many complications. A transjugular intrahepatic portosystemic stent shunt (TIPS) is a metal prosthesis that has been shown to be very effective in lowering sinusoidal portal pressure, and therefore is effective in the management of complications of cirrhosis, especially those related to portal hypertensive bleeding and sodium and water retention. In patients with acute variceal bleeding not responding to pharmacologic and endoscopic treatments, a reduction of the hepatic venous pressure gradient to < 12 mmHg or by > 20% with TIPS has been shown to be effective in controlling the acute bleed and in preventing rebleeding. For stable patients whose acute variceal bleed is controlled, TIPS is equal to combined beta-blocker and band ligation in the prevention of recurrent variceal bleed. TIPS is also more effective than large volume paracentesis in the control of refractory ascites, and may confer a survival advantage over repeated large volume paracentesis. TIPS has also been used in the management of other complications related to portal hypertension including ectopic varices, hepatic hydrothorax, and hepatorenal syndrome with some success, but experience is still rather limited. Miscellaneous uses include treatment of Budd Chiari Syndrome, portal hypertensive gastropathy and hepatopulmonary syndrome. Careful patient selection is vital to a successful outcome, as patients with severe liver dysfunction tend to die post-TIPS despite a functioning shunt. All patients who require a TIPS for treatment of complications of cirrhosis should be referred for consideration of liver transplant.     

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.     

Transjugular Intrahepatic Portosystemic Shunt: A Successful Treatment for Hepatopulmonary Syndrome

Hepatopulmonary syndrome is a well described complication of chronic liver disease. Though uncommon, it carries a high morbidity and mortality. The pathogenesis of the syndrome has not been clearly defined. Portal hypertension seems to play a crucial role in the pathogenesis of the syndrome, probably by enhancing nitric oxide production. As yet, no pharmacological therapy has been proven effective. Many reports of successful reversal of the syndrome after liver transplantation have been published. We report a patient with hepatopulmonary syndrome who showed a significant and durable (4 months') improvement in his symptoms, arterial oxygenation, and intrapulmonary shunts, as calculated by radionuclide studies after transjugular intrahepatic portosystemic shunt placement. Transjugular intrahepatic portosystemic shunt may represent a durable treatment option for patients with hepatopulmonary syndrome.     

Transjugular intrahepatic portosystemic shunt in cavernomatous portal vein occlusion

A 72-year-old patient with liver cirrhosis and cavernomatous portal vein occlusion presented with refractory ascites. We treated the patient with transjugular intrahepatic portosystemic shunt: transjugular transhepatic puncture of an intrahepatic hilar collateral vein was performed; transjugular intrahepatic portosystemic shunt was created between the right hepatic vein and the patent superior mesenteric vein using this hilar collateral vein as the connecting pathway. The ascites was resolved and the patient remains asymptomatic, while shunt patency is maintained 16 months after the intervention.     

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.     

Middle hepatic vein reconstruction of graft for a patient with intrahepatic portosystemic shunt

Middle hepatic vein reconstructions for modified right liver grafts in living donor liver transplantation provide satisfactory results. We report a patient who had undergone transjugular intrahepatic portosystemic shunt before living donor liver transplantation, in which the middle hepatic vein was reconstructed using a preserved great saphenous vein. A 41-year-old Japanese man with a 5-year history of alcoholic liver cirrhosis and esophageal varices was admitted to our hospital for living donor liver transplantation. He had undergone endoscopic variceal ligations and transjugular intrahepatic portosystemic shunt for esophageal variceal bleeding, and ascites. He had living donor liver transplantation, which was performed using his sister's right lobe without the middle hepatic vein. The recipient's estimated standard liver volume calculated by abdominal computer-assisted tomography was 1166 mL. The exact weight of the donor's right lobe was 507 g, which was equivalent to 44% of the recipient's standard liver volume. At bench surgery, the middle hepatic vein was reconstructed using a preserved great saphenous vein, which was cut in 2 strips to make a thicker tube graft by suturing, and subsequently, the newly made tube graft end was anastomosed to V5 and V8 branches of the graft. The metallic stent for transjugular intrahepatic portosystemic shunt buried in the recipient's right hepatic vein was removed with the right hepatic vein. The other end of the saphenous tube graft was anastomosed to the right anterior aspect of the vena cava. Stumps of the middle and left hepatic veins were oversewn. Postoperative blood flow in the graft and the reconstructed hepatic veins has been satisfactory with normal liver functions.     

Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome

BACKGROUND: We report our experience with management of patients with Budd Chiari syndrome over the past two decades. In 1996 we described a novel approach involving recanalisation of hepatic veins by combined percutaneous and transvenous approaches. This was incorporated into an algorithm published in 1999 in which our preferred treatment for all cases of Budd Chiari syndrome with short segment occlusion or stenosis of the hepatic veins involves recanalisation of the hepatic veins by transvenous or combined percutaneous-transvenous approaches. In symptomatic Budd Chiari syndrome where recanalisation is not possible, we perform transjugular intrahepatic portosystemic shunts (TIPS) because TIPS decompresses the portal circulation directly in an adjustable way. In this series of patients with Budd Chiari syndrome treated with radiological interventions alone, we assess their medium term outcome using two independent objective prognostic indices. METHODS: We retrospectively studied 61 patients with non-malignant Budd Chiari syndrome treated by radiological intervention alone in our centre. RESULTS: Actuarial survival for the entire cohort at one year and five years was 94% and 87%, respectively. Survival of our patients with mild disease (according to the Murad classification) was 100% at one year and at five years, with intermediate disease severity 94% at one year and 86% at five years, and with severe disease 85% at one year and 77% at five years. CONCLUSION: Management of Budd Chiari syndrome by interventional radiology resulted in excellent medium term survival for patients in all categories of disease severity.     

Transjugular intrahepatic portosystemic shunt for hepatorenal syndrome: A systematic review and meta-analysis

BackgroundHepatorenal syndrome is a severe complication of advanced liver diseases with a dismal prognosis.AimsThis systematic review and meta-analysis aims to explore the efficacy and safety of transjugular intrahepatic portosystemic shunt for the treatment of hepatorenal syndrome.MethodPublications were searched via PubMed and EMBASE databases. The pooled proportion and mean difference were calculated by using a random-effect model.ResultsNine publications were included, in which 128 patients with hepatorenal syndrome were treated with transjugular intrahepatic portosystemic shunt. The pooled short-term and 1-year survival rates were 72% and 47% in type 1 hepatorenal syndrome and 86% and 64% in type 2 hepatorenal syndrome. No lethal procedure-related complications were observed. The pooled rate of hepatic encephalopathy after transjugular intrahepatic portosystemic shunt was 49%. The pooled rate of renal function improvement after transjugular intrahepatic portosystemic shunt was 93% in type 1 hepatorenal syndrome and 83% in any type of hepatorenal syndrome. After transjugular intrahepatic portosystemic shunt, serum creatinine, blood urea nitrogen, serum sodium, sodium excretion, and urine volume were significantly improved; by comparison, serum bilirubin slightly increased, but the difference was not statistically significant.ConclusionLimited evidence suggested a potential survival benefit of transjugular intrahepatic portosystemic shunt in patients with hepatorenal syndrome but with a high incidence of hepatic encephalopathy.     

Transjugular intrahepatic portosystemic shunt: a case report of rescue management of unrestrainable variceal bleeding in a pregnant woman.

Liver cirrhosis complications in pregnant women are frequent and death rate secondary to variceal bleeding is relevant. Both sclerotherapy and banding ligation seem to be safe procedures in pregnancy; when bleeding is not arrested endoscopically an emergency transjugular intrahepatic portosystemic shunt should be considered, but data regarding pregnant cirrhotic women are scarce. We describe the case of a pregnant woman at 14 weeks of gestation who underwent management of acute variceal bleeding by transjugular intrahepatic portosystemic shunt. Transjugular intrahepatic portosystemic shunt may represent a rescue treatment for failed attempts of band ligation or sclerotherapy.     

Transjugular intrahepatic portosystemic shunt for treatment of bleeding ectopic varices with portal hypertension

PURPOSE: In the setting of hepatic failure and portal hypertension, hemorrhage from stomal and rectal varices is a well-described problem. It has recently been suggested that transjugular intrahepatic portosystemic shunting may be useful in the therapy of bleeding from parastomal or anorectal varices in patients unresponsive to conservative therapy. METHODS: We retrospectively review our institution's experience of five patients with parastomal varices and seven patients with anorectal varices who underwent transjugular intrahepatic portosystemic shunting for hemorrhage refractory to conservative management between 1994 and 1998. RESULTS: The study group consisted of four Child's A, five Child's B, and three Child's C patients. The mean age of the patients was 60.3 (range, 37-85) years. Mean follow-up was 15 (range, 5-27) months. The mean portosystemic pressure gradient before transjugular intrahepatic portosystemic shunting was 17.4+/-3.1 mm Hg. After transjugular intrahepatic portosystemic shunting, the mean portosystemic pressure gradient was reduced to 5.8+/-1.8 mm Hg (P<0.05). Transjugular intrahepatic portosystemic shunting were successful in complete resolution of bleeding in all patients. Three patients had encephalopathic changes after transjugular intrahepatic portosystemic shunting. Two patients died within 30 days of transjugular intrahepatic portosystemic shunting of causes unrelated to the procedure. Four patients required shunt revision within one year of placement. CONCLUSION: The transjugular intrahepatic portosystemic shunting procedure is an effective modality in the therapy of cirrhotic patients with bleeding stomal or anorectal varices unresponsive to conservative management. There is an acceptable procedure-related morbidity and mortality.     

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.     

Transjugular intrahepatic portosystemic shunt for intractable posthepatectomy ascites

We report two cases of transjugular intrahepatic portosystemic shunt for control of intractable ascites after resection of cirrhotic livers. The first case was a 46-year-old male who had undergone right lobectomy of the liver for a small hepatocellular carcinoma. His liver function had recovered within a week after the operation, but ascites drainage of 1-4 L/day persisted for more than a month despite vigorous medical therapy. We performed transjugular intrahepatic portosystemic shunt on the 49th postoperative day and the pressure gradient between the right atrium and the left portal vein was reduced to from 21 mmHg to 6 mmHg. Thereafter, ascites became responsive to diuretic therapy and was well controlled without complication. Second case of a 54-year-old male patient who had undergone left lateral segmentectomy due to a small hepatocellular carcinoma presented intractable ascites of 1-3 L/day, which was also effectively controlled after transjugular intrahepatic portosystemic shunt performed on the 34th postoperative day, though there was an episode of hepatic encephalopathy stage 1. Based on our limited experience, hepatectomized patients suffering from prolonged intractable ascites despite a favorable profile of liver function may be candidates for transjugular intrahepatic portosystemic shunt with an acceptable risk of hepatic failure and procedure-related complication.     

Efficacy and safety of transjugular intrahepatic portosystemic shunt in the treatment of nongastric extraesophageal variceal bleeding

OBJECTIVES: Although esophageal varices are the most common site of variceal bleeding, extraesophageal varices cause up to 30% of variceal bleeding. Unlike esophageal variceal bleeding, the experience in management of extraesophageal variceal bleeding, especially nongastric extraesophageal variceal bleeding is limited, and there are no established guidelines for treatment of nongastric extraesophageal variceal bleeding. This study aims to provide experience in treatment of nongastric extraesophageal variceal bleeding with transjugular intrahepatic portosystemic shunt in a tertiary medical center. METHODS: We retrospectively reviewed all cases, admitted or transferred to Emory University Hospital, with extraesophageal variceal bleeding who had transjugular intrahepatic portosystemic shunt as the final resolution to control bleeding over a period of 4 years, from January 1999 to January 2003. We also compared the outcomes after transjugular intrahepatic portosystemic shunt for bleeding from gastric varices and nongastric extraesophageal varices. RESULTS: Forty-one patients (33 gastric varices and 8 nongastric extraesophageal varices) with extraesophageal variceal bleeding who had transjugular intrahepatic portosystemic shunt performed were identified in this study period. Bleeding was controlled immediately in 90% (37/41) of those patients. The mortality was 7% (3/41). The rebleeding rate was 10% (4/41). Encephalopathy occurred in 24% (10/41) of the patients. Patients with gastric varices bleeding appeared to have more advanced liver disease than patients with nongastric extraesophageal varices bleeding. The outcomes after transjugular intrahepatic portosystemic shunt for bleeding from gastric varices and nongastric extraesophageal varices were similar. CONCLUSIONS: Transjugular intrahepatic portosystemic shunt is an effective and safe treatment of extraesophageal variceal bleeding, including bleeding from gastric varices and nongastric extraesophageal varices.     

Fluoroscopy-induced radiodermatitis after transjugular intrahepatic portosystemic shunt

Transjugular intrahepatic portosystemic shunt is a nonsurgical procedure used to manage the complications of portal hypertension. This report describes three cases of fluoroscopy-induced radiodermatitis after transjugular intrahepatic portosystemic shunt and reviews the characteristics and treatment of radiation-induced skin reactions.     

Surgical portosystemic shunts in the era of TIPS and liver transplantation are still relevant

BackgroundThe surgical portosystemic shunts (PSS) are a time-proven modality for treating portal hypertension. Recently, in the era of liver transplantation and the transjugular intrahepatic portosystemic shunts (TIPS), use of the PSS has declined.ObjectivesThis study was conducted to evaluate changes in practice, referral patterns, and short- and longterm outcomes of the use of the surgical PSS before and after the introduction of the Model for End-stage Liver Disease (MELD).MethodsA retrospective analysis of 47 patients undergoing PSS between 1996 and 2011 in a single university hospital was conducted.ResultsSubgroups of patients with cirrhosis (53%), Budd–Chiari syndrome (13%), portal vein thrombosis (PVT) (26%), and other pathologies (9%) differed significantly with respect to shunt type, Child–Pugh class, MELD score and perioperative mortality. Perioperative mortality at 60 days was 15%. Five-year survival was 68% (median: 70 months); 5-year shunt patency was 97%. Survival was best in patients with PVT and worst in those with Budd–Chiari syndrome compared to other subgroups. Patency was better in the subgroups of patients with cirrhosis and other pathologies compared with the PVT subgroup. Substantial changes in referral patterns coincided with the adoption of the MELD in 2002, with decreases in the incidence of cirrhosis and variceal bleeding, and increases in non-cirrhotics and hypercoagulopathy.ConclusionsAlthough the spectrum of diseases benefiting from surgical PSS has changed, surgical shunts continue to constitute an important addition to the surgical armamentarium. Selected subgroups with variceal bleeding in well-compensated cirrhosis and PVT benefit from the excellent longterm patency offered by the surgical PSS.