Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Review Dig Surg 2006;23:358–369 DOI: 10.1159/000097897 Current Role of Portosystemic Shunt Surgery in the Management of Hepatic Venous Outflow Obstruction Dinesh Singhal a, b Steve de Castro a Neerav Goyal b Dirk J. Gouma a A. Chaudhary b Thomas M. van Gulik a a Department of Surgery, Academic Medical Center, Amsterdam, The Netherlands, and b Department of Surgical Gastroenterology, Sir Ganga Ram Hospital, New Delhi, India reported from specialized centers. The main limitation for PSS is the reported perioperative mortality of 10–20% and a declining technical expertise for such surgery. Liver trans- plantation with disease-specific 5-year survival between 50 and 95% is presently the treatment of choice for patients with fulminant presentation, end-stage liver disease (ESLD), unshuntable anatomy or decompensation after PSS. TIPS may be preferable for sick patients with acute presentation with isolated hepatic vein thrombosis or as a temporizing measure for those with ESLD awaiting transplantation. The drawback of TIPS is late shunt dysfunction that occurs in more than 50% of patients at 1 year. Conclusions: Due to rarity of the disorder there is a lack of trials comparing the different treatment modalities. Hence, the current treatment recommendations are based on retrospective studies. In a select group of HVOO patients (subacute presentation with preserved liver function), PSS remains the treatment of choice with excellent long-term results. Copyright © 2006 S. Karger AG, Basel Introduction Hepatic venous outflow obstruction (HVOO) is a rare syndrome that results from blockage of the venous drain- age of the liver due to hepatic vein thrombosis and/or in- ferior vena cava (IVC) obstruction. The clinical manifes- Key Words Budd-Chiari syndrome  Hepatic vein thrombosis  Shunts  Portosystemic shunts  Transjugular intrahepatic portosystemic shunt  Angioplasty Abstract Background: Hepatic venous outflow obstruction (HVOO) is a rare disorder that occurs predominantly due to a hyperco- agulable state. The syndrome may result from hepatic vein obstruction, inferior vena cava obstruction or a combination of both and manifests with post-sinusoidal portal hyperten- sion. The presentation may be fulminant with poor progno- sis or as either acute, subacute or chronic forms with rela- tively better prognosis. Portosystemic shunt surgery (PSS) has thus far been the mainstay of treatment for HVOO. How- ever, over the last decade, transjugular intrahepatic porto- systemic shunt (TIPS) and liver transplantation have emerged as viable options. This review aims to evaluate the available treatment options and the current relevance of PSS for the management of HVOO. Methods: A literature review on in- vestigations and treatment was performed using Medline and additional library searches. Results: Portosystemic shunts form the mainstay of treatment for patients with sub- acute presentation (preserved liver function) with medically intractable ascites or recurrent variceal hemorrhage. Excel- lent results with 5-year survival of more than 90% have been Published online: December 11, 2006 Prof. T.M. van Gulik Department of Surgery, Academic Medical Center Meibergdreef 9 NL–1105 AZ Amsterdam (The Netherlands) Tel. +31 20 566 2166, Fax +31 20 691 4858, E-Mail t.m.vangulik@amc.uva.nl © 2006 S. Karger AG, Basel 0253–4886/06/0236–0358$23.50/0 Accessible online at: www.karger.com/dsu