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%.     

Budd–Chiari syndrome: illustrated review of current management

Budd–Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is discussed. Particular attention is paid to interventional and surgical aspects of management. The review is augmented by images, which provide a clinical corollary to the text.     

Multiple thrombophilic factors in a patient with Budd–Chiari syndrome

Myeloproliferative disorders are the main cause of Budd–Chiari syndrome in western countries. Inherited or acquired thrombophilic factors have also been implicated. A novel mutation of the prothrombin gene (G→A20210) has only been described in a few cases of Budd–Chiari syndrome so far. Venous thrombosis is often the result of multiple concomitant thrombophilic factors. We report the case of a patient with essential thrombocythemia and Budd–Chiari syndrome in which heterozygosity for both factor V Leiden and the mutation G20210A of the prothrombin gene were identified.     

Haemodynamic response to abdominal decompression in acute Budd–Chiari syndrome

Background: Intra‐abdominal hypertension (IAH) and abdominal compartment syndrome commonly occur in patients with liver disease. Aims: We compared haemodynamic variables pre‐ and post‐abdominal decompression in patients with acute Budd–Chiari syndrome (BCS) and patients with chronic liver disease (CLD), ascites and IAH. Methods: Patients with IAH admitted to the Liver ICU, King's College Hospital were studied. Transpulmonary thermodilution cardiac output (CO) monitoring was performed with the PiCCO^® system. Results: Ten patients with decompensated BCS (median age 39 years, 20–52) and eight patients with CLD (59 years, 33–65) and tense ascites requiring paracentesis were studied. Intra‐abdominal pressure (IAP) was raised in both groups pre‐intervention (BSC 23 mmHg, 17–40; CLD 26, 20–40). Intrathoracic blood volume (ITBVI) was persistently low in the BCS group (632 ml/m², 453–924) despite volume resuscitation. Post‐intervention, reduction in IAP was noted in both groups (BCS P<0.001, CLD P<0.0001). The ITBVI increased (P=0.001) in the BCS group only. An increase in cardiac index (CI) and stroke volume index (SVI) was noted in both groups (BCS: CI P=0.003, SVI: P=0.007; CLD: CI P=0.005, SVI P=0.02). The central venous pressure did not change in either group and did not correlate with markers of flow (CI, SVI) or IAP. Both groups demonstrated an inverse relationship between IAP, CI and SVI. Conclusion: Patients with BCS and IAH have evidence of central hypovolaemia. In addition to raised IAP, hepatic venous obstruction and caudate lobe hypertrophy limit venous return in patients with BCS. Reduction in IAP and re‐establishment of caval flow restores preload with improvement in CO.     

Milestones in the discovery of Budd‐Chiari syndrome

In 1845, George Budd published a brief report regarding three patients who developed an obstruction of the hepatic veins. The condition has never been reported before, and was related to sepsis and alcoholism. Fifty‐three years later, Hans Chiari postulated that syphilis was causing the obstruction of the hepatic veins, and enriched the debate with clinical and pathological correlations. Following the hypothesis on the ‘phlebitis obliterans’, several authors proposed other pathophysiological explanations including congenital causes, chronic trauma and exogenous toxins. RG Parker, in 1959, first recognized the relationship between obstruction of hepatic veins and thrombophilic conditions such as polycythaemia vera, pregnancy and hormonal therapy. Based on that, anticoagulant treatment was attempted, but with unsatisfactory outcome. We need to wait until the mid 1980s to see a widespread adoption of anticoagulants, with a consequent improvement of patients' survival. The fear of haemorrhagic events in patients with liver disease discouraged this therapeutic approach, and other surgical interventions (mainly port‐systemic shunts) were conceived, but with high morbidity and mortality. The first liver transplantation in 1976 and the first trans‐jugular intra‐hepatic porto‐systemic shunt in 1993 represented two major cornerstones in the management of Budd‐Chiari syndrome (BCS). Such progresses allowed modifying the treatment of BCS until the modern concept of stepwise therapy. The present review thoroughly reviews the major landmarks in the discovery, treatment and clinical management of patients with BCS.     

Budd–Chiari syndrome due to membranous obstruction of inferior vena cava in a patient with Behcet's disease

Budd–chiari syndrome is a well known complication of Behcet's disease. All cases of Behcet's disease with Budd–Chiari syndrome that have been reported in the literature had thrombosis of hepatic veins or inferior vena cava. We describe a 33‐year‐old woman with Behcet's disease who developed Budd–Chiari syndrome due to a membranous web of the inferior vena cava.     

Transcatheter closure of a large ASD from the femoral vein in a patient with Budd–Chiari syndrome

Anomalies of the inferior vena cava are rare in patients with isolated atrial septal defect (ASD). When present, they usually preclude successful transcatheter closure of the ASD using the femoral route. We report a case of a large secundum ASD in a 3‐year‐old child, who had a chronic Budd–Chiari syndrome incidentally detected on the cardiac catheterization table. We were able to successfully deploy the device from the femoral route itself using a collateral channel to reach the heart and then using the pulmonary vein deployment technique to successfully close the defect. © 2012 Wiley Periodicals, Inc.     

Long‐term outcome of a covered vs. uncovered transjugular intrahepatic portosystemic shunt in Budd–Chiari syndrome

Background: The clinical outcome of a covered vs. uncovered transjugular intrahepatic portosystemic shunt (TIPS) for patients with Budd–Chiari syndrome (BCS) is as yet largely unknown. Objectives: To compare patency rates of bare and polytetrafluoroethylene (PTFE)‐covered stents, and to investigate clinical outcome using four prognostic indices [Child–Pugh score, Rotterdam BCS index, modified Clichy score and Model for End‐Stage Liver Disease (MELD)]. Methods: Consecutive patients with BCS who had undergone TIPS between January 1994 and March 2006 were evaluated in a retrospective review in a single centre. Results: Twenty‐three TIPS procedures were performed on 16 patients. The primary patency rate at 2 years was 12% using bare and 56% using covered stents (P=0.09). We found marked clinical improvement at 3 months post‐TIPS as determined by a drop in median Child–Pugh score (10–7, P=0.04), Rotterdam BCS index (1.90–0.83, P=0.02) and modified Clichy score (7.77–2.94, P=0.003), but not in MELD (18.91–17.42, P=0.9). Survival at 1 and 3 years post‐TIPS was 80% (95% CI: 59–100%) and 72% (95% CI: 48–96%). Four patients (25%) died and one required liver transplantation. Conclusions: A transjugular intrahepatic portosystemic shunt using PTFE‐covered stents shows better patency rates than bare stents in BCS. Moreover, TIPS leads to an improvement in important prognostic indicators for the survival of patients with BCS.