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Budd-Chiari Syndrome
1Liver Study Unit, Section of Gastroenterology, Dept. of Internal Medicine, The University of Chicago Hospitals and Clinics, 5841 S. Maryland Ave., MC 4076, Chicago, IL 60637, USA.
Insights
Budd-Chiari syndrome treatment involves managing hepatic vein or vena cava obstruction. Therapies range from less invasive procedures like angioplasty and shunts to surgical options and liver transplantation for severe cases.
Area of Science:
- Gastroenterology and Hepatology
- Vascular Surgery
- Interventional Radiology
Background:
- Budd-Chiari syndrome involves thrombotic or non-thrombotic occlusion of hepatic veins and/or vena cava.
- Effective management aims to relieve venous obstruction and preserve liver function.
Purpose of the Study:
- To review and summarize current diagnostic and therapeutic options for Budd-Chiari syndrome.
- To discuss the efficacy and indications for various treatment modalities.
Main Methods:
- Review of existing literature on Budd-Chiari syndrome management.
- Analysis of different therapeutic approaches, including medical, interventional, surgical, and transplantation.
Main Results:
- Less invasive therapies like percutaneous transluminal angioplasty (PTA) and transjugular intrahepatic portosystemic shunts (TIPS) show promise for short-term outcomes.
- Surgical interventions and liver transplantation are reserved for complex or advanced cases.
- Outcomes vary, with potential for liver function improvement but also risk of deterioration in patients with compromised hepatic reserve.
Conclusions:
- A spectrum of treatment options exists for Budd-Chiari syndrome, tailored to disease presentation and severity.
- Further research is needed to establish long-term efficacy of interventional procedures like TIPS.
- Liver transplantation remains a critical option for fulminant hepatic failure or end-stage liver disease.
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.