Budd-Chiari syndrome (BCS) or hepatic venous outflow obstruction, is a rare and complex clinical entity, with diverse aetiology and a wide range of presentations. Its rarity and heterogeneity create significant challenges for evidence-based clinical decision-making, particularly in the even more infrequent setting of acute BCS presentation. Here we present the case of a young woman with acute, severe BCS, where treatment alternatives were limited due to a significant IVC thrombus burden. A clearly defined acute onset allowed the prompt use of systemic thrombolysis, conducted in consultation with a multi-disciplinary team of clinicians, with close monitoring for both treatment response and complications. Her treatment resulted in resolution of hepatic vein occlusion and prevention of the potentially significant clinical sequelae. We review the literature and discuss current opinion in the management of BCS.
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Duke Univ, Duke Clin Res Inst, Durham, NC 27715 USA
Princess Alexandra Hosp, Dept Gastroenterol, Brisbane, Qld 4102, AustraliaDuke Univ, Duke Clin Res Inst, Durham, NC 27715 USA
Clark, Paul J.
Slaughter, Richard E.
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Prince Charles Hosp, Chair Med Imaging, Brisbane, Qld 4032, AustraliaDuke Univ, Duke Clin Res Inst, Durham, NC 27715 USA
Slaughter, Richard E.
Radford, Dorothy J.
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Prince Charles Hosp, Dept Cardiol, Adult Congenital Heart Dis Unit, Brisbane, Qld 4032, AustraliaDuke Univ, Duke Clin Res Inst, Durham, NC 27715 USA
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Paul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Coilly, Audrey
Potier, Pascal
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St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, France
De La Source Hosp, Reg Hosp Orleans, Dept Gastroenterol & Hepatol, 14 Ave Hop, F-45100 Orleans, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Potier, Pascal
Broue, Pierre
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St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, France
Univ Hosp Toulouse, Pediat Hepatol Unit, 9 Pl Lange, F-31300 Toulouse, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Broue, Pierre
Kounis, Ilias
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Paul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Kounis, Ilias
Valla, Dominique
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St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, France
Beaujon Hosp AP HP, Dept Hepatol, 100 Blvd Gen Leclerc, F-92118 Clichy, France
European Reference Network ERN Rare Liver, Reference Ctr Vasc Liver Dis, Hamburg, GermanyPaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Valla, Dominique
Hillaire, Sophie
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Foch Hosp, Dept Internal Med, 40 Rue Worth, F-92150 Suresnes, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Hillaire, Sophie
Lambert, Vincent
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Gen Med, 46 Ave Yolande Aragon, F-49100 Angers, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Lambert, Vincent
Dutheil, Danielle
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St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, France
Beaujon Hosp, Dept Hepatol, Assoc Patients Vasc Liver Dis AMVF, 100 Blvd Gen Leclerc, F-92118 Clichy, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Dutheil, Danielle
Hernandez-Gea, Virginia
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Univ Barcelona, Barcelona Hepat Hemodynam Lab, Liver Unit, Hosp Clin,Inst Invest Biomed August Pi & Sunyer I, Barcelona, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Hlth Care Provider European Reference Network onR, Barcelona, SpainPaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Hernandez-Gea, Virginia
Plessier, Aurelie
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St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, France
Beaujon Hosp AP HP, Dept Hepatol, 100 Blvd Gen Leclerc, F-92118 Clichy, France
European Reference Network ERN Rare Liver, Reference Ctr Vasc Liver Dis, Hamburg, GermanyPaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Plessier, Aurelie
Vilgrain, Valerie
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St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, France
Beaujon Hosp AP HP, Dept Hepatol, 100 Blvd Gen Leclerc, F-92118 Clichy, France
Beaujon Hosp, AP HP, Dept Radiol, 100 Blvd Gen Leclerc, F-92118 Clichy, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France
Vilgrain, Valerie
Bureau, Christophe
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St Antoine Hosp, APHP, French Network Rare Liver Dis FILFOIE, 184 Rue Faubourg St Antoine, F-75012 Paris, France
Univ Hosp Toulouse, Rangueil Hosp, Dept Gastroenterol & Hepatol, 1 Ave Prof Jean Poulhes, F-31400 Toulouse, FrancePaul Brousse Hosp APHP, Hepatobiliary Ctr, 12 Ave Paul Vaillant Couturier, F-94800 Villejuif, France