Excellent long-term outcomes of endovascular treatment in budd-chiari syndrome with hepatic veins involvement: A STROBE-compliant article.

This study aimed to evaluate the long-term efficacy and safety of percutaneous transhepatic balloon angioplasty (PTBA) and transjugular intrahepatic portosystemic stent-shunt (TIPSS) in the treatment of Budd-Chiari syndrome (BCS) with hepatic veins involvement. Between June 2008 and August 2016, a total of 60 BCS patients with hepatic vein involvement in our department were enrolled in this study. Thirty-three cases underwent hepatic vein balloon angioplasty in PTBA Group and 27 cases underwent TIPSS. Data were retrospectively collected, and follow-up observations were performed. TIPSS Group showed significantly higher thrombotic/segmental obstruction and peripheral stenosis/obstruction compared with PTBA Group. The success rates were 93.9% and 100.0% in PTBA Group and TIPSS Group, respectively. The mean portal vein pressure decreased significantly after stenting. Except for 1 patient died from repeated hemorrhage, other sever complications had not been observed in both group. Twenty-six patients and 21 patients were clinically cured in PTBA Group and TIPSS Group, respectively. The primary patency rates were 89.7%, 79.3%, and 79.3% for short-term, mid-term and longterm in PTBA Group, which were significantly higher than TIPSS Group for long-term follow up. The second patency rates were 100.0%, 96.6% and 96.6% for short-term, mid-term and long-term in PTBA Group, which were similar to TIPSS Group (P = 1.0000). In conclusion, PTBA and TIPSS are safe and effective in the treatment of BCS with hepatic veins involvement, with an excellent long-term patency rate of hepatic vein and TIPSS shunt. TIPSS can be used to treat patients with all 3 hepatic veins lesion and failure PTBA.

Budd-Chiari syndrome: imaging review.

Budd-Chiari syndrome (BCS), also known as hepatic venous outflow tract obstruction includes a group of conditions characterized by obstruction to the outflow of blood from the liver secondary to involvement of one or more hepatic veins (HVs), inferior vena cava (IVC) or the right atrium. There are a number of conditions that lead to BCS-ranging from hypercoagulable states to malignancies. In up to 25% patients, no underlying disorder is identified. Diagnosis of BCS is based on a combination of clinical and imaging features. A major part of the literature in BCS has been devoted to interventions; however, a detailed description of various imaging manifestations of BCS is lacking. In this review, we highlight the importance of various imaging modalities in the diagnosis of BCS.

The epidemiology of Budd-Chiari syndrome in France.

INTRODUCTION: Epidemiological data is lacking on primary Budd-Chiari syndrome (BCS) in France. METHODS: Two approaches were used: (1) A nationwide survey in specialized liver units for French adults. (2) A query of the French database of discharge diagnoses screening to identify incident cases in adults. BCS associated with cancer, alcoholic/viral cirrhosis, or occurring after liver transplantation were classified as secondary. RESULTS: Approach (1) 178 primary BCS were identified (prevalence 4.04 per million inhabitants (pmi)), of which 30 were incident (incidence 0.68 pmi). Mean age was 40 ±â€¯14 yrs. Risk factors included myeloproliferative neoplasms (MPN) (48%), oral contraceptives (35%) and factor V Leiden (16%). None were identified in 21% of patients, ≥2 risk factors in 25%. BMI was higher in the group without any risk factor (25.7 kg/m2 vs 23.7 kg/m2, p < 0.001). Approach (2) 110 incident primary BCS were admitted to French hospitals (incidence 2.17 pmi). MPN was less common (30%) and inflammatory local factors predominated (39%). CONCLUSION: The entity of primary BCS as recorded in French liver units is 3 times less common than the entity recorded as nonmalignant hepatic vein obstruction in the hospital discharge database. The former entity is mostly related to MPN whereas the latter with abdominal inflammatory diseases.

Risk factors of recurrence among 471 Chinese patients with Budd-Chiari syndrome.

BACKGROUND AND AIM: Budd-Chiari syndrome (BCS) is a rare form of vascular disease. There is limited literature available regarding the prognosis of this disease. The aim of this study was to characterize the cumulative recurrence rates and to investigate the risk factors of recurrence in Chinese patients with BCS. METHODS: Four hundred and seventy-one patients were diagnosed as having BCS in the Affiliated Hospital of Xuzhou Medical College (Jiangsu, China) between January 2008 and December 2012. Follow-ups were conducted by phone calls or correspondence. Cumulative recurrence rates were assessed with the Kaplan-Meier curves. Independent risk factors of recurrence were calculated with the Cox's proportional hazards regression model. RESULTS: Four hundred and twenty-five patients with BCS had complete follow-up data, in which 24 patients died, 98 patients had recurrence, with the median duration of follow-up being 19.3 months (range 3 to 61.4). The cumulative 1-, 2-, 3-, 4- and 5-year recurrence rates were 12%, 22%, 27%, 35% and 42%, respectively. Univariable and multivariable Cox's proportional hazards regression models showed that the risk factors of recurrence include: age ≤ 30 years (HR=2.261, 95% CI: 1.412-3.621), differentiated typology (hepatic vein type: HR=1.885, 95% CI: 1.045-3.402; combined type: HR=2.088, 95% CI: 1.233-3.536), elevated lactate dehydrogenase (LDH) levels (HR=1.125, 95% CI: 1.101-1.212) and the Child-Pugh class B/C (B: HR=1.758, 95% CI: 1.057-2.926; C: HR=2.626, 95% CI: 1.396-4.940). CONCLUSIONS: Regardless of thrombophilia and haematological causes, exceptionally found in Chinese patients, the 5-year recurrence rate of BCS was as high as 42%. Age ≤ 30 years, hepatic vein type, combined type, increased LDH levels and the Child-Pugh class B/C were independent predictors of BCS recurrence.

Clinical and histological features of different types of Budd-Chiari syndrome: a comparison of 4 cases.

Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction. In this report, we present 4 cases of BCS with complete and incomplete obstruction of the inferior vena cava (IVC) and hepatic vein (HV). Each case showed different and unique features of liver damage, which were attributed to the site and degree of obstruction. Interestingly, improved liver functions such as increased serum albumin levels, decreased hyaluronic acid levels and a normal indocyanine green clearance test were evident within 1 month of the balloon angioplasty. Pericellular fibrosis and hypervascular regenerative nodules were also reversible after obstruction removal. Therefore, it is very important to manage this rare disease before it progresses to liver cirrhosis.

Update on the classification, assessment of prognosis and therapy of Budd-Chiari syndrome.

Budd-Chiari syndrome (BCS) occurs as a result of 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. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The traditional classification of BCS--as fulminant, acute or chronic--is not prognostically useful. This makes assessing the benefit of therapy difficult, especially as there is no evidence from randomized studies. This article highlights advances in the prognosis and therapy of BCS. Identification of the site of venous obstruction has a major effect on prognosis. Portal-vein thrombosis occurs in 20-30% of cases, and acute presentation of BCS reflects an acute or chronic syndrome in 60% of BCS cases. BCS can be diagnosed and treated on a single occasion in the setting of the radiology department, with hepatic venography, transjugular liver biopsy, retrograde CO2 portography and inferior vena cava pressure measurements performed simultaneously with therapies such as dilation or stenting of webs in the inferior vena cava or hepatic veins, and placement of transjugular intrahepatic portosystemic shunts. Disruption of a portal vein thrombus can also be done during the same session. Surgical shunts have been superseded by the use of transjugular intrahepatic portosystemic shunts. Liver transplantation is reserved for fulminant and progressive chronic forms of BCS. Anticoagulation therapy must be used routinely, before and after specific therapy, regardless of whether a thrombophilic disorder is diagnosed.

Surgical treatment of 1360 cases of Budd-Chiari syndrome: 20-year experience.

BACKGROUND: Budd-Chiari syndrome (BCS) is a disease caused by blood flow obstruction of the main hepatic veins (MHVs) and/or the outlet of the inferior vena cava (IVC), characterized by retrohepatic portal hypertension (PHT) and/or IVC hypertension. In the past decade, over 3000 cases of BCS have been reported in China. This study was to sum up our 20-year experience in surgical treatment of BCS and to investigate its pathological classification and principles of surgery. METHODS: The data from 1360 BCS patients were analyzed retrospectively. RESULTS: Four types (6 subtypes) were classified according to IVC angiography and hepatovenography: type Ia (594 patients), type Ib (123), type II (292), type IIIa (237), type IIIb (112), and type IV (2). Surgical procedures included: improved splenopneumopexy (265 cases), finger or balloon membranotomy (407), radical resection of membrane and thrombus (275), IVC bypass (88: cavocaval transflow 71 cases, and cavoatrial transflow 17 cases), mesocaval C-shape shunt (192), splenocaval shunt (32), splenoatrial shunt (23), splenojugular shunt (57), mesoatrial shunt (8), and combined methods (6), including plenal-cavoatrial shunt (4), and mesocavoatrial shunt (2), splenorenal shunt (4), mesojugular shunt (2), and other methods (1). The perioperative death rate and the complication rate after operation was 3.09% (42/1360) and 14.8% (201/1360) respectively. 885 cases were followed up from 9 months to 15 years (average 6.8+/-1.2 years. The 791 (89.4%) of 885 patients were successfully treated, 61 patients (6.89%) had a recurrence, and 33 died. CONCLUSION: Surgical treatment of BCS is dependent on a correct diagnosis and classification of the disease.

[Staged surgery in the treatment of Budd-Chiari's syndrome of mixed type].

9 patients with Budd-Chiari's syndrome of mixed type were operated upon. Among them 3 cases were treated by percutaneous transluminal angioplasty using balloon catheters, and 1 to 2 weeks after followed by Hassab's procedure and Linton's procedure. Two cases were treated by incision and plasty of IVC, and 1 month after followed by Hassab's procedure and Linton's procedure. The remaining 4 were treated by prehepatic cavoatrial shunt, succeeded by Hassab's procedure and Linton's procedure one month after. 6 to 24 months' follow up of the 9 cases revealed satisfactory results.

Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome.

Severe hepatic venous outflow obstruction and its manifestations often are recorded under the label "Budd-Chiari syndrome." Unfortunately, this label is ambiguous; it does not clearly identify the site of the lesion (hepatic veins versus inferior vena cava), its morphologic features (thrombotic versus nonthrombotic), or its cause. In the literature, implied or expressed definitions vary. Use of a standardized topographic and pathogenetic classification of hepatic venous outflow obstruction would enable investigators to group patients with comparable conditions, as required for therapeutic trials, prognostic evaluations, and studies of pathogenetic pathways. Review of our own cases revealed that hepatic venous outflow obstruction involving large hepatic veins is usually thrombotic and that isolated obstruction of the inferior vena cava or of small hepatic veins is usually nonthrombotic. Application of such a classification seems feasible and may yield useful results.

Approach to the spectrum of Budd-Chiari syndrome: which patients require portal decompression?

Budd-Chiari syndrome (occlusion of the hepatic veins) represents a spectrum disorder. From 1974 to 1984, 20 patients with the syndrome were managed. Eleven required shunt surgery (Group 1) and 5 were managed with nonshunt therapy (Groups 2 and 3). Results have been good. Retrospective review of the liver biopsy specimens showed that Group 1 patients had a greater degree of zone 3 necrosis than Group 2 and 3 patients. We submit that presence of zone 3 necrosis on an initial liver biopsy specimen may define the failing liver of Budd-Chiari syndrome that requires conversion of the portal vein to an outflow tract by shunting.