Budd-Chiari Syndrome: Illustrated Review of Current Management

John D. Horton; Francisco L. San Miguel; Jorge A. Ortiz

Disclosures

Liver International. 2008;28(4):455-466. 

In This Article

Interventional Techniques and Transjugular Intrahepatic Portosystemic Shunt

In recent years, percutaneous interventions have played an increasing role in the management of patients with BCS. Indeed, most institutions are more likely to possess the capability to perform TIPS than to employ a surgeon with the expertise to perform a mesenteric-systemic shunt.[9] Consequently, TIPS has become the first option when a shunting procedure is indicated.[30] Catheter-directed thrombolytic therapy, angioplasty and stent placement can be effective in the acute setting,[16] while TIPS may be employed in the acute or chronic setting.

A recent 10 patient case series and literature review by Sharma concluded that systemic thrombolytic therapy is of little value and that catheter-directed thrombolysis is more efficacious for acute thrombus that is not completely occlusive. The authors also felt that tissue plasminogen activator is the preferred agent and that it should be delivered just proximal to or within the thrombus.[31]

Angioplasty and stenting are additional adjuncts that may be used. Angioplasty often suffers from high reocclusion rates. Thus, placement of stents in the IVC or hepatic veins has been recommended (Fig. 4).[31] A study out of China recently reported a case series of 115 in which stents were placed in the IVC and hepatic veins with success rates of 94% and 87% respectively. Patency reached 90% after a mean follow-up of more than 45 months.[32] The proportion of patients with membranous occlusion, which may be more amenable to angioplasty and stenting, was much more prevalent in this population (57%) than would be expected in most Western institutions.

Stent Placement (arrow) Across an Inferior Vena Cava (IVC) Stenosis in a Patient With Budd-Chiari Syndrome (BCS).

Angioplasty and stenting may offer certain advantages to patients who suffer from BCS after liver transplantation. The aetiology in this subset of patients is usually secondary to technical problems.[33] Assuming hepatic function has not been lost, correction of the technical issue may be accomplished by percutaneous means and may obviate the need for more invasive procedures.[34] Clinical improvement and long-term durability have been reported in small case series.[8]

Unfortunately, most patients do not present acutely and their disease is often not amenable to thrombolytics, angioplasty or stenting. For these patients who present weeks to months after formation of hepatic vein thrombus, TIPS has become an attractive option in the elective and emergent situations.[16] TIPS effectively decompresses the portal system and may serve as a bridge to transplant. The first report of TIPS for the treatment of BCS was in 1993,[35] and since that time it has become a useful tool in the clinician's armamentarium. Extended TIPS with or without thrombolysis can be used in cases of BCS coupled with portal vein thrombosis.[30] Two series with a total of 34 patients showed improved patency and less dysfunction with polytetrafluoroethylene (PTFE)-covered stents compared with bare stents.[35,36] Other series using TIPS in BCS report eventual transplant in 10-40% of patients.[16] Misplacement or migration of TIPS into the portal vein is not uncommon (Fig. 5).[9,37] This particular complication can have adverse consequences if the patient goes on for transplantation.

Coronal Magnetic Resonance Imaging (MRI) Showing (a) Cranial Migration of Transjugular Intrahepatic Portosystemic Shunt (TIPS) (arrow) and Resulting Impingement on the Inferior Vena Cava (IVC). (b) The Impingement of the IVC Was Relieved By Placing a Stent (white arrow) in the IVC Adjacent to the TIPS (black arrow).

A case series of 61 patients who underwent TIPS and/or hepatic vein recanalization with angioplasty and stenting showed excellent survival. However, 40 patients required repeated radiological interventions. An important conclusion from this study was that short-length stenosis of hepatic veins is under-recognized and percutaneous recanalization is under-utilized in the management of patients with BCS. This study suggested that when stratified by disease severity, interventional radiology techniques may have the most impact on the sickest patients (compared with survival in patients treated with surgical shunts). Forty-four patients treated initially with surgical shunts, transplantation or medical therapy alone were not included for analysis in this study.[26]

Interpretation of the literature in this area can be difficult. Results from a case series in Asia may not apply to the patient population in Western countries because the incidence of membranous obstruction is much higher in the Asian series. Also, technology evolves quickly and today's stents used for TIPS and other percutaneous interventions may have very different properties than those from only a few years ago. As stated before, no prospective trials in patients with BCS exist and conclusions from any published report should be viewed with caution.

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