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Budd–Chiari Syndrome Revisited: 38 Years’ Experience with Surgical Portal Decompression

  • 2011 SSAT Plenary Presentation
  • Published:
Journal of Gastrointestinal Surgery

Abstract

Objective

Ten years ago, we reported our results with what remains as the largest clinical experience with surgical portal decompression for Budd–Chiari syndrome (BCS) in the West. Since then, our series has expanded to 77 patients, and there has been an explosion of interest in and publications about BCS. The objectives of this study are to assess the validity of our observations and conclusions regarding BCS reported 10 years ago by expansion of our series of patients and observations of outcomes over an additional decade of close follow-up.

Methods

Seventy-seven patients with BCS were allocated to three groups: group I, 39 had hepatic vein occlusion alone, treated by side-to-side portacaval shunt (SSPCS); group II, 26 had inferior vena cava occlusion treated by mesoatrial shunt in eight and combined SSPCS and cavoatrial shunt (CAS) in 18; and group III, 12 had decompensated cirrhosis too late for portal decompression who were listed for liver transplantation (LT). An extensive diagnostic workup included angiography with pressure measurements and needle liver biopsy. Follow-up was 100%, lasting 5–38 years.

Results

In group I, long-term survival is 95% with 36 free of ascites, leading lives of good quality 5–38 years post-SSPCS. In group II, mesoatrial shunt was discontinued after 1990 because of a high failure rate, but combined SSPCS-CAS has resulted in 100% survival for 5–25 years. In group III, six patients (50%) are alive and well for more than 5 years post-LT. Serial liver biopsies following portal decompression have shown long-term reversal of the lesions of BCS.

Conclusions

Long-term survival following portal decompression of BCS in the West has not been equaled by any other form of therapy, medical or surgical. It is imperative to perform surgical portal decompression early in the course of BCS in order to avoid irreversible liver damage.

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Acknowledgment

This study was supported by grant 1R01 AM17103 from the National Institutes of Health and a grant from the Surgical Education and Research Foundation [501(c)(3)].

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Authors and Affiliations

  1. Department of Surgery, University of California San Diego Medical Center, 200 West Arbor Drive, San Diego, CA, 92103-8999, USA

    Marshall J. Orloff, Pat O. Daily & Barbara Girard

  2. Departments of Medicine/Gastroenterology, University of California San Diego Medical Center, San Diego, CA, USA

    Jon I. Isenberg & Henry O. Wheeler

Authors
  1. Marshall J. Orloff
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  2. Jon I. Isenberg
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Correspondence to Marshall J. Orloff.

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Discussant

Jean Nicolas Vauthey (Houston, TX): Dr. Orloff and his team should be congratulated for reporting an update of their experience with the treatment of Budd–Chiari syndrome. The study confirms the importance of early surgical intervention with portocaval shunt or combined portocaval shunt and cavoatrial shunt. In the last 200 papers on Budd–Chiari in the Medline, it is striking to see that large series are uncommon and most of the literature consists in small series or case reports. As such, Dr. Orloff’s series is an exception to the rule and another example of the value of updated databases relying on large experience.

I have two questions for the authors:

1. Some series have proposed a classification of the Budd–Chiari syndrome based on anatomical finding or on disease extent. Can these classifications be used and safely applied for the treatment of Budd–Chiari syndrome?

2. Medical, minimally invasive, and surgical treatment modalities are competing for the treatment of this syndrome. Patients are often treated based on patterns of referral. Is there a role for medical treatment or minimally invasive procedures in these patients?

3. What is the exact role of transjugular intrahepatic portosystemic shunts in Budd–Chiari syndrome? Are portosystemic shunts and liver transplantation the only definitive therapies available today in Budd–Chiari syndrome?

Closing Discussant

Dr. Marshall J. Orloff: Thank you, Dr. Vauthey, for your comments and questions. In response to your first question, I must emphasize, as our data demonstrate, that the anatomical location of hepatic venous outflow obstruction determines what treatment is indicated. When occlusion is confined to the hepatic veins alone and the disease is acute or subacute and has not progressed to the point of cirrhosis and hepatic decompensation, treatment by surgical side-to-side portacaval shunt is highly effective. Ninety-five percent of such patients in our series were alive for follow-up lasting 5–38 years, were free of ascites, and had lives of good quality. On the other hand, when occlusion involves the IVC, the combination procedure that we have developed is required. It consists of a side-to-side portacaval shunt plus a cavoatrial shunt. In our series, the combined procedure has resulted in 100% survival for follow-up of 5–25 years.

In response to your second question, our experience demonstrates that there is no definitive role for medical treatment or minimally invasive procedures in BCS. Recently, some physicians in Europe have recommended a stepwise approach to treatment beginning with a trial of anticoagulants, then a trial of angioplasty or thrombolysis and stenting, then a trial of TIPS, and finally liver transplantation. The stepwise plan carries with it the danger of permitting liver damage to become advanced and irreversible. Our experience shows that it is imperative to perform portal decompression as soon as possible after the diagnosis of BCS has been made.

In response to your third question regarding use of TIPS in BCS, in recent years, TIPS has been used with considerable frequency in BCS, mainly in Europe. As you know, TIPS is a side-to-side portacaval shunt inserted percutaneously under radiographic control and, as such, is based on the same rationale as surgical side-to-side portacaval shunt. The main problem with TIPS is its lack of durability, that is, a high incidence of TIPS occlusion requiring re-intervention and ultimate failure, even with use of the recently introduced polytetrafluorethylene-covered stents. In contrast, surgical portacaval shunts remained permanently patent in all but one patient in our series. In summary, our long experience has shown that surgical portal decompression in BCS has resulted in a survival rate and quality of life unequaled by TIPS or any other form of treatment used in countries in the West.

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Orloff, M.J., Isenberg, J.I., Wheeler, H.O. et al. Budd–Chiari Syndrome Revisited: 38 Years’ Experience with Surgical Portal Decompression. J Gastrointest Surg 16, 286–300 (2012). https://doi.org/10.1007/s11605-011-1738-9

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