Budd-Chiari syndrome

Abstract

Many options are available to diagnose and treat patients with the Budd-Chiari syndrome who present with either thrombotic or non-thrombotic occlusion of the major hepatic veins and or vena cava. The goal of therapy is to alleviate venous obstruction and to preserve hepatic function. Low-sodium diets, diuretics, and therapeutic paracentesis are generally ineffective, except for the rare patient who presents with volume overload and incomplete hepatic venous occlusion. Anticoagulants and thrombolytics may be appropriate for selected patients with acute thrombotic venous obstruction. Percutaneous transluminal angioplasty (PTA) of hepatic venous stenoses or caval webs with or without placement of intraluminal stents yield excellent short-term results, but additional studies are warranted to assess long-term efficacy. Transjugular intrahepatic portosystemic shunts (TIPS) may be effective for patients with subacute or chronic disease and ascites refractory to sodium restriction and diuretics. Intrahepatic stents may also serve as a bridge to transplantation for selected patients presenting with fulminant hepatic failure consequent to hepatic venous occlusion. Additional studies will be necessary to assess the role of TIPS in the armamentarium of therapies for patients with the Budd-Chiari syndrome. Decompressive shunts, reconstruction of the vena cava and hepatic venous ostia, transatrial membranotomy, and dorsocranial resection of the liver with hepatoatrial anastomosis are appropriate options for patients with acute or subacute disease who are not candidates for, or fail less invasive therapies. The majority of patients benefit with improvement in liver function tests, ascites, and liver histology; however, hepatic function may deteriorate in patients with marginal reserve. Liver transplantation is reserved for patients with Budd-Chiari syndrome who present with fulminant hepatic failure or end-stage liver disease with portal hypertensive complications. Transplantation is also appropriate for patients who deteriorate after failed attempts at surgical shunting.

Imaging of Budd-Chiari syndrome

“…1). Either part or all of the hepatic venous system or IVC may be thrombosed or occluded [16,17]. There may be visualised discontinuity between flow in the IVC and hepatic veins and sometimes reversal of flow may be identified within the hepatic veins.…”

Budd–Chiari‐like syndrome in a dog with a chondrosarcoma of the thoracic wall

“…Several surgical management options are used including creation of a portal or mesenteric to systemic shunt, placement of stents within the liver or within the inferior vena cava, and vessel reconstruction. Liver transplantation is performed in some cases 12,23 . Advances in treatment options have improved survival rates in humans 19 .…”

“…Liver transplantation is performed in some cases. 12,23 Advances in treatment options have improved survival rates in humans. 19 These include catheter-directed thrombolysis, percutaneous transluminal angioplasty of stenoses or membranous webs with or without the placement of intraluminal stents, transjugular intrahe-patic portosystemic shunt, and liver transplantation.…”

Mechanisms of Acute Liver Failure

“…Depending on the progression of the disease, Budd-Chiari syndrome may result in ALF when sudden closing of all three main liver veins occurs. Typically, acute Budd-Chiari syndrome presents with ascites, abdominal pain, jaundice, and hepatomegaly (22).…”

“…Budd-Chiari syndrome is frequently associated with primary myeloproliferative disorders, a factor V Leiden mutation, anticardiolipin antibodies, and protein C and S deficiencies that increase the risk of thrombotic complications (21)(22)(23). In general, the course of disease in Budd-Chiari syndrome leads to liver transplantation.…”