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

Surgical Shunts

The role of surgical shunts in the treatment of BCS is a controversial topic. A wide range of perioperative mortality has been reported (0-50%).[3] However, long-term survival rates (5-14 years) after various shunting procedures have been reported to be as high as 90%.[16] There are multiple factors that make it difficult to interpret the literature on this subject. Studies that compare surgical shunts to medical or minimally invasive techniques are often difficult to interpret because they are usually retrospective, may have significant time bias and, maybe most importantly, the sickest patients are usually managed nonoperatively.[38] Also, in recent years, experience with these procedures has begun to dwindle. For example, chief residents at a large academic centre such as Johns Hopkins only performed four portosystemic decompression surgeries between 1995 and 1999.[9]

Shunting procedures are indicated in patients with reversible liver injury. However, identifying this population of patients is not straightforward.[39] Anatomic considerations such as extensive IVC thrombus may make TIPS difficult and favour placement of a surgical shunt. While several cases of BCS with portal vein and/or mesenteric vein thrombosis have been successfully managed with TIPS and thrombolysis,[30] open surgical intervention may be required.

Selection of which surgical shunt to perform requires consideration of several factors. For patients without IVC obstruction, the portocaval or mesocaval shunts are reasonable options. Previous portocaval shunts can make the hilar dissection during transplantation particularly challenging. In patients with caudate lobe hypertrophy (Fig. 1), portocaval shunts may be difficult and even require caudate lobe resection before construction.[9] Orloff et al. [40]reported great success with portocaval shunts with 31 of 32 patients alive at 3.5-27 years after surgery. Unfortunately, their success has not been replicated by others.[9]

For patients with IVC obstruction and/or an infrahepatic to right atrium pressure gradient of >20mmHg, mesocaval and portocaval shunts may not effectively decompress the liver. Some authors report improved long-term outcomes with a pressure gradient of greater than 10mmHg between the portal vein and IVC.[40] The most important pressure gradient is not between the infra and suprahepatic vena cava, but between the two vascular structures that are to be connected.[3] Consequently, many authors consider mesoatrial or cavoatrial shunts in these cases (Fig. 6).[9] While the primary patency rate of mesoatrial shunts is relatively low, 5-year survival has been acceptable.[9,41]

Intraoperative Photo Taken During a Cavoatrial Shunt Procedure Showing the Anastomosis of the Graft to the Infrahepatic Vena Cava (arrow). The liver is retracted medially and the graft is then brought posteriorly through the diaphragm and into the right hemithorax.

Wang [2]reported good 1-, 3- and 5-year patency rates for mesoatrial (90.7%, 77.1%, 61.1%) and cavoatrial (97.2%, 86.0%, 79.8%) shunts. Orloff reported 18 cases of BCS with IVC and hepatic vein occlusion. In their early experience, eight of these patients were treated with mesoatrial shunts. Five of these patients died after the graft thrombosed. The authors then began to use a combined side-to-side portocaval shunt and cavoatrial shunt in these patients. Results were much better with all 10 patients surviving to their last follow-up (mean follow-up 9 years).[40]

The literature is unclear with regard to which shunt should be used in any particular situation. No prospective studies exist. Retrospective studies are heterogeneous and often include data from three or more types of surgical shunts. Many comparative studies have either no control group or a small control group.[3] Clinical judgment and technical expertise often dominate the decision-making process.

Portocaval shunts require disconnection during liver transplantation. This can result in increased operative time and blood loss. Alternatively, mesocaval shunts are not part of the operative field during transplantation, can be divided easily and may simplify the procedure by providing portal and systemic decompression during intraoperative venovenous bypass through a single femoral vein cannula.[9]

Two case series with a total of 73 patients underwent surgical shunting procedures before transplantation. Both of these studies showed worse outcomes in the patients who had a portocaval shunt. Additionally, the larger of the two studies showed a 5-year survival advantage of mesocaval shunts compared with patients without previous shunt procedures (95% vs. 65%).[42,43]

In Langlet's extensive review of the literature on surgical shunting, only two studies assessed survival after correcting for prognostic factors. Neither of these studies could demonstrate any favourable effects of surgical shunts on survival. Thus, the review argues that the late beneficial effects of relieving hepatic congestion may be balanced by the high early mortality of the procedure.[3] Murad's multivariate analysis stratified 205 patients with BCS treated by a variety of modalities into three classes and found that surgical shunting and TIPS was associated with increased mortality as the interval between diagnosis and shunting lengthened. The study also showed a trend toward improved survival in class II patients (RR, 0.63; P=0.29).[44]

Another surgical option is radical resection with anatomic reconstruction.[45] This approach may be more applicable in Asian countries, where the aetiology is often IVC or hepatic vein membranes.

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