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.     

Portal vein aneurysm associated with Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt: A case report

A 65-year-old woman with Budd-Chiari syndrome(BCS) presented with right upper quadrant pain.A computed tomography(CT) scan showed a saccular aneurysm located at the extrahepatic portal vein main branch measuring 3.2 cm in height and 2.5 cm × 2.4 cm in diameter.The aneurysm was thought to be associatedwith BCS as there was no preceding history of trauma and it had not been present on Doppler ultrasound examination performed 3 years previously.Because of increasing pain and concern for complications due to aneurysm size, the decision was made to relieve the hepatic venous outflow obstruction.Transjugular intrahepatic portosystemic shunt(TIPS) was created without complications.She had complete resolution of her abdominal pain within 2 d and remained asymptomatic after 1 year of follow-up.CT scans obtained after TIPS showed that the aneurysm had decreased in size to 2.4 cm in height and 2.0 cm × 1.9 cm in diameter at 3 mo, and had further decreased to 1.9 cm in height and 1.6 cm × 1.5 cm in diameter at 1 year.     

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.     

Paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome: therapeutic challenge with bone marrow transplantation, transjugular intrahepatic portosystemic shunt, and vena cava stent

Stenosis of inferior vena cava can be a cause of or a consequence of Budd-Chiari syndrome (BCS). However, its occurrence after transjugular intrahepatic portosystemic shunt (TIPS) insertion has only been twice reported. We report the case of a 23-year-old man who presented BCS of the three suprahepatic veins. The cause of BCS was paroxysmal nocturnal hemoglobinuria, which was treated by bone marrow transplantation. A few months later, it was necessary to insert a TIPS because of refractory ascites and severe denutrition. However, refractory ascites was persistent and esophageal varices bleeding occurred. A TIPS desobstruction was needed, and during this angiography a stenosis of the suprahepatic vena cava was found and was treated with dilatation and stent. In a few weeks, the patient's clinical and nutritional state improved and, after 9 months hospitalization, the patient was discharged. One year later, this patient had no symptoms with an excellent permeability of TIPS and vena cava stent. This case report confirms that TIPS is a good treatment for BCS, but stenosis of inferior vena cava can occur after such a treatment.     

Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt

BACKGROUND: The long-term outcome of Budd-Chiari syndrome (BCS) with transjugular intrahepatic portosystemic shunts (TIPS) is not well studied. To address this, the records of 47 consecutive patients with BCS evaluated in one center from January 1989 to April 2004, were analyzed. RESULTS: Seven patients with liver tumors were excluded from analyses. Eleven patients had Bechet's disease, 14 had thrombophiliac disorders, four had myeloproliferative diseases and 11 patients had other or unknown causes. The site of block was hepatic vein in 16 patients, in the suprhepatic inferior vena cava in 19 and not known in five. The majority of patients (21/40; 52.5%) presented with subacute disease with massive ascites and abdominal pain as the dominant manifestations. Eight patients with membranes or segemental block were treated with transluminal angiopalsty, and six were treated with clinical and biochemical recovery. The TIPS was placed through a transcaval puncture in eight patients with progressive liver disease who were on medical therapy and had thrombosis limited to hepatic veins. One patient bled from portal vein puncture, which was managed by placing stent across the punctured site. The TIPS was very effective in decreasing portal pressure gradient, improving synthetic functions, reducing transaminase levels and controlling ascites. Five patients had TIPS dysfunction needing revision. In two patients it was difficult to maintain TIPS patency due to repeated TIPS dysfunction. However, both these patients were asymptomatic with normal liver function tests. Long-term follow up revealed that patients with TIPS had significantly better survival than those treated with medical therapy alone (log-rank test, P = 0.04). In a multivariate Cox-model analysis four variables, namely, more florid presentation, male sex, no treatment with TIPS and increasing Child-Pugh-Turcotte score, adversely affected the survival. CONCLUSIONS: Budd-Chiari syndrome needs an individualized multidisciplinary approach and TIPS is indicated in a subgroup of patients with progressive liver disease. It is safe, feasible and improves survival.     

Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt

AIM:To evaluate long-term complications and survival in patients with Budd-Chiari syndrome (BCS) referred to a Danish transjugular intrahepatic portosystemic shunt (TIPS) centre.METHODS:Twenty-one consecutive patients from 1997-2008 were retrospectively included [15 women and 6 men,median age 40 years (range 17-66 years)].Eighteen Danish patients came from the 1.8 million catchment population of Aarhus University Hospital and three patients were referred from Scandinavian hospitals.Management consisted of tests for underlying haematological,endocrinological,or hypercoagulative disorders parallel to initiation of specific treatment of BCS.RESULTS:BCS was mainly caused by thrombophilic (33%) or myeloproliferative (19%) disorders.Fortythree percents had symptoms for less than one week with ascites as the most prevalent finding.Fourteen (67%) were treated with TIPS and 7 (33%) were manageable with treatment of the underlying condition and diuretics.The median follow-up time for the TIPS-treated patients was 50 mo (range 15-117 mo),and none required subsequent liver transplantation.Ascites control was achieved in all TIPS patients with a marked reduction in the dose of diuretics.A total of 14 TIPS revisions were needed,mostly of uncovered stents.Two died during follow-up:One non-TIPS patient worsened after 6 mo and died in relation to transplantation,and one TIPS patient died 4 years after the TIPS-procedure,unrelated to BCS.CONCLUSION:In our BCS cohort TIPS-treated patients have near-complete survival,reduced need for diuretics and compared to historical data a reduced need for liver transplantation.     

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.     

Budd-Chiari syndrome in a patient with paroxysmal nocturnal hemoglobinuria

A case of Budd-Chiari syndrome developing in a 29-year-old male with paroxysmal nocturnal hemoglobinuria (PNH) is reported. The patient represents the fifth reported case of proven hepatic vein thrombosis and Budd-Chiari syndrome in a patient with PNH, and is the first person to survive more than 3 years after onset of symptoms.     

Spondylodiscitis associated with recurrent Serratia bacteremia due to a transjugular intrahepatic portosystemic shunt (TIPS): a case report.

We report a case of spondylodiscitis caused by multiresistant Serratia marcescens in a cirrhotic patient who had several Serratia bacteremias after the placement of a transjugular intrahepatic portosystemic shunt (TIPS) device. We concluded that an endovascular stent that can not be removed makes management of recurrent bacteremia difficult. Furthermore, back pain due to bacteremia is indicative of spondylodiscitis. Serratia marcescens can be an aggressive pathogen, causing spinal infection.     

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.     

经颈内静脉肝内门体分流术治疗肝肾综合征临床研究

[目的]观察经颈内静脉肝内门体分流术(TIPS)治疗肝肾综合征(HRS)的临床疗效,探讨其可能的作用机制。[方法]10例确诊为HRS患者行TIPS,分别于术前及术后测门静脉压力、门静脉内径及血流速度、尿钠排泄、尿量、血肌酐(Cr)、尿素氮(BUN)及血Cr清除率;术前及术后7 d测定肾素(PRA)、血管紧张素Ⅱ(AT-Ⅱ)、醛固酮(ALD)水平。[结果]TIPS术后门静脉压力、门静脉内径及血流速度分别为(2.3±0.3)kPa、(1.26±0.04)cm、(44.2±14.5)cm/s,与术前比较均P<0.01。BUN、血Cr、尿钠排泄、血Cr清除率及尿量手术前后差异有统计学意义(均P<0.01)。术后PRA、AT-Ⅱ、ALD分别为(67.12±25.28)、(138.02±58.54)、(597.37±284.25)ng/L,与术前比较,P<0.01,<0.05,<0.05。[结论]TIPS治疗HRS近期疗效明显,其作用可能与血容量的增加和肾素-血管紧张素-醛固酮系统改变有关。     

阵发性睡眠性血红蛋白尿合并脑静脉窦血栓形成1例并文献复习

阵发性睡眠性血红蛋白尿（paroxysmalnocturnalhemoglobinuria,PNH）是一种后天获得性造血干细胞克隆缺陷性疾病,可表现为溶血性贫血、血红蛋白尿和静脉血栓形成。脑静脉窦血栓形成（cerebral venous sinus thrombosis, CVST ）是PNH的一种少见但较严重的并发症。文章报道1例PNH合并CVST患者,并对相关文献进行了复习。对于伴有CVST 危险因素且表现为头痛、颅内压升高等神经系统症状的患者,应及早行颅脑影像学检查明确诊断,积极给予抗凝、脱水、祛除病因、控制并发症等方面的治疗。     

Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome

Paroxysmal nocturnal hemoglobinuria (PNH) is associated with a high risk of thrombosis, particularly in the peripheral, cerebral, and abdominal veins. We report a patient with an occlusion of the hepatic veins and a slit shape narrowing of the cava inferior consistent with the Budd-Chiari syndrome in whom intravenous fibrinolytic therapy with recombinant tissue plasminogen activator (rt-PA) was applied. Systemic rt-PA was given in a dose of 25 mg rt-PA over 3 h and 25 mg rt-PA as constant intravenous infusion over the next 21 h leading to an incomplete recanalization. The same protocol was applied again 2 days later, resulting in a complete recanalization of the hepatic veins and the vena cava inferior. Our case shows that exclusive systemic application of rt-PA can result in full anatomic and clinical restoration.     

Late polymicrobial transjugular intrahepatic portosystemic shunt infection in a liver transplant patient: A case report

BACKGROUNDInfection of a transjugular intrahepatic portosystemic shunt (TIPS) stent is a rare and serious complication that most commonly occurs during TIPS creation and revision. Patients typically present with recurrent bacteremia due to shunt occlusion or vegetation. To date there are approximately 58 cases reported. We present a patient diagnosed with late polymicrobial TIPS infection five years following TIPS creation.CASE SUMMARYA 63-year-old female status-post liver transplant with recurrent cirrhosis and portal hypertension presented with sepsis and recurrent extended-spectrum beta-lactamase Escherichia coli bacteremia. Computed tomography of the abdomen revealed an occluded TIPS with thrombus extension into the distal right portal vein, and focal thickening of the cecum and ascending colon. Colonoscopy revealed patchy ulcers in these areas with histopathology demonstrating ulcerated colonic mucosa with fibrinopurulent exudate. Shunt thrombectomy and revision revealed infected-appearing thrombus. Patient initially cleared her infection with antibacterial therapy and TIPS revision; however, soon after, she developed Enterobacter cloacae bacteremia and Candida glabrata and C. albicans fungemia with recurrent TIPS thrombosis. She remained on antifungal therapy indefinitely and later developed vancomycin-resistant Enterococcus faecium with recurrent TIPS thrombosis. The option of liver re-transplant for removal of the infected TIPS was not offered given her critical illness and complex shunt anatomy. The patient became intolerant to linezolid and elected hospice care.CONCLUSIONClinicians should be aware that TIPS superinfection may occur as long as five years following TIPS creation in an immunocompromised patient.     

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.     

Spontaneous bacteremia due to Salmonella hadar in liver cirrhosis with transjugular intrahepatic portosystemic shunt

Bacteriemia is a frequent infection in patients with hepatic cirrhosis, which is associated with a high mortality. Spontaneous bacteriemia is generally caused by gramnegative bacilli. We present an episode of Salmonella hadar's spontaneous bacteriemia in a patient with cirrhosis of the liver and transjugular intrahepatic portosystemic shunt. The role of the prosthesis in the pathogenesis of bacteriemia in hepatic cirrhosis is discussed.     

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

Budd-Chiari syndrome(BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt(TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.