Stent angioplasty of narrowed portocaval shunt in Budd Chiari syndrome: a case report
© Doctor et al; licensee BioMed Central Ltd. 2009
Received: 01 December 2008
Accepted: 01 January 2009
Published: 01 January 2009
Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from an obstruction to the outflow of blood from the liver. Early decompression is needed to prevent liver dysfunction and death. Radiological intervention includes angioplasty of stenosis and webs and the placement of transjugular intrahepatic portosystemic shunts (TIPPS). Side-to-side portacaval shunt (SSPCS) remains the gold standard for achieving good long-term results.
A 37-year old lady underwent side-to-side portacaval shunt for Budd Chiari syndrome. She had early shunt blockage and this was successfully treated with the placement of a metallic stent across the shunt.
At five years, she remains asymptomatic, with normal liver functions, no ascites, and normal flow through the stent on Colour Doppler examination.
Budd-Chiari syndrome is characterized by hepatic venous outflow obstruction, which often leads to death as a result of portal hypertension and liver failure. Patients with membranous occlusion of the major hepatic veins can be treated by percutaneous placement of a metallic stent via a transjugular or transhepatic approach . In patients with a significant caval obstruction caused by a hypertrophied caudate lobe, a metallic vascular stent can be placed in the narrowed tract of the inferior vena cava, before shunt surgery, by means of a transfemoral or transjugular venous approach . Side to side portacaval shunts have excellent long-term results and remain the procedure of choice in some units . Stents can also be used to maintain the patency of surgical shunts that have been made to bypass the obstruction to the blood flow from the splanchnic circulation to the inferior vena cava via the liver. We report a long term follow up of stent angioplasty of a narrowed porta caval shunt in a patient with Budd Chiari Syndrome.
A thirty seven year old female presented with a history of progressive distension of the abdomen with pedal edema and yellow discolouration of the eyes of two weeks duration. On examination she was found to be icteric with an enlarged, firm liver and gross ascites. Liver function tests revealed a conjugated hyperbilirubinemia with mildly deranged transaminases and alkaline phosphatase. Ultrasonography (USG) with Colour Doppler showed an enlarged liver with a hypertrophied caudate lobe, a 1.1 cm portal vein with hepatopetal flow, an occluded right hepatic vein with middle and left hepatic veins patent only in their proximal parts and a patent but narrowed inferior vena cava, compressed by the caudate lobe, together with free fluid in the abdomen. Splenic and superior mesenteric veins were normal. Liver biopsy confirmed the diagnosis of Budd Chiari syndrome. Prothrombotic workup did not reveal any obvious cause for the Budd Chiari syndrome.
The portocaval gradient dropped to < 5 mmHg post stenting. Subsequently she was anticoagulated with warfarin. The patient has been on regular follow up since then and is asymptomatic for five years.
BCS is characterized by manifestations of portal hypertension, inferior vena cava occlusion or both . It produces liver damage due to intense congestion, leading to development of cirrhosis. To prevent congestion, various nonsurgical and surgical procedures have been described.
A variety of surgical procedures can be used for decompression of the liver, including mesoatrial, mesocaval and portocaval shunts and liver transplantation. If there is no thrombosis in the inferior vena cava, a side-to-side portocaval shunt (SSPCS) is the procedure of choice in the early and middle stages of the disease . Direct portocaval shunts have a thrombosis rate of 0.2%, compared with occlusion rates 24–53% for mesocaval shunts with synthetic grafts. However, autologous internal jugular vein grafts have patency rates comparable with those of portocaval shunts . Mesoatrial shunts are preferred if there is a greater than 75% narrowing of the vena cava . However, the patency rate of mesoatrial shunts is low, Slakey et al.  reporting a primary shunt patency rate of only 46%. To improve the shunt patency Orloff et al.  introduced a high-flow combination shunt, consisting of a side-to side portocaval shunt and a cavoatrial shunt. Results of this combined form were promising. Orthotopic liver transplantation is indicated in patients with chronic BCS who have cirrhosis and patients with a failed portosystemic shunt procedure. It has a 5-year survival rate of 69% . Blockage of the shunts performed to decompress the liver can be a life-threatening event and is usually associated with excessive variceal hemorrhage. Recently percutaneous interventional techniques, including angioplasty, fibrinolysis, Self expanding metallic stents (SEMS) and TIPPS, have gained attention in the treatment of BCS and shunt occlusion . Angioplasty has a high success rate in BCS due to congenital obstruction. For restenosis, balloon dilatation can be performed. As angioplasty simulates intimal hyperplasia, the number of balloon dilatations must be limited . Xu et al.  advocated the use of metallic stents for such angioplasty failures. SEMS placement makes the dilatation more definitive.
The drop in venous pressure is more marked and it keeps the vessel patent for a longer time than angioplasty. But growth of a hyperplastic intima around the wire may reduce the diameter of the inferior vena cava. The stent may also cross the outlet of the hepatic vein, which results in an increase of turbulent flow and may lead to thrombosis . TIPPS can be performed in BCS patients to improve the clinical condition while waiting for orthotopic liver transplantation. The TIPPS procedure is relatively safe and effective but its long-term patency is limited. Fortunately TIPPS stenosis responds well to reinterventions such as balloon dilatation and stents . There are limited cases in the literature of percutaneous procedures secondary to a failed shunt, as most would become eligible for transplant. In our patient, we placed a SEMS in a narrowed portocaval shunt. As an interventional procedure, SEMS produced a good long term result with clinical improvement and patency. Therefore SEMS could be the procedure of choice in occluded portocaval shunts. We propose the use of SEMS as a salvage procedure for blocked side to side portocaval H-grafts before consideration of more radical procedures like liver transplantation.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Transjugular Intrahepatic Portosystemic Shunt
Side-to-side portacaval shunt
Budd Chiari Syndrome
Self expanding metallic stent
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