Management of Budd?Chiari Syndrome

Abstract

Thrombotic occlusion of the hepatic veins leads to liver dysfunction and liver failure requiring liver transplantation in advanced cases. The cause for the occlusion of the hepatic veins is not completely understood. However, several underlying conditions such as polycytemia, factor V Leiden mutation, and protein C and S deficiency are found in these patients. We here report our single-center experience with 18 consecutive patients with Budd-Chiari Syndrome (BCS) who were treated at our institution between August 1992 and June 2003. Twelve patients underwent liver transplantation, three patients received stents into the hepatic veins or vena cava, another patient was treated with TIPSS (transjugular intrahepatic postosystemic stent shunt), and one patient underwent surgical mesocaval shunting. Three patients, among those the patient with TIPSS, were put on anticoagulant therapy and are scheduled for liver transplantation. We outline the indication for an approach tailored to the stage of the disease and the adaption of the procedures with the deterioration of clinical conditions. Surgical aspects and postoperative management with a focus on liver transplantation are outlined. We conclude from our observations that the management of BCS requires an approach that exhausts conservative approaches until clinical conditions deteriorate with respect to portal hypertension or liver function. Conservative management, i.e., interventional and supportive medical therapy, has been used up to 8 years in our series, until the time for liver transplantation is reached. Liver transplantation for BCS had more complications than transplantation for other liver diseases in our series. Therefore, we propose to keep liver function stable using interventional techniques to maintain venous outflow. If venous outflow cannot be interventionally restored and liver function deteriorates or cirrhosis develops during this time course, liver transplantation is the therapy of choice.

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

“…It is an uncommon disorder occurring in 1/100 000 of the general population worldwide (2). Nonetheless, if left untreated, BCS may lead to liver dysfunction and liver failure requiring liver transplantation in advanced cases (3). The prognosis of patients with BCS is difficult to predict.…”

The Role of Intravascular Intervention in the Management of Budd-Chiari Syndrome

“…In cases of interventional treatment for hepatic venoocclusive lesions, the second hepatic hilum-occlusive HV should be reopened [9,10]. Open the connection and use a stent between the IVC and HV under the internal jugular vein (9/11) (Fig.…”

Budd–Chiari syndrome from an advanced practice nurse perspective

“…A study of 18 patients with BCS by Ruh et al (2005) revealed that myoproliferative disorders (groups of conditions that cause overproduction of blood cells, i.e., platelets, white blood cells, and red blood cells in the bone marrow) were common risk factors among the patients. This observation was corroborated by Slakey, Klein, Venbrux, and Cameron (2001), who noted that 22 of 54 reported cases involved myeloproliferative disorders.…”

“…Pregnancy is another risk factor, especially when the patient has an established thrombophilia history (Ruh et al, 2005). Behcet’s disease, an autoimmune disorder with recurrent thrombosis and vasculitis, and paroxysmal nocturnal hemoglubinuria are two additional established causes of BCS.…”