Budd-Chiari Syndrome: The Western Perspective

  • Aurélie Plessier
  • Audrey Payancé
  • Dominique Valla
Chapter

Abstract

Primary Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction from small hepatic veins to the inferior vena cava (IVC), due to thrombosis or fibrous stenosis of these veins. In Western countries, one or multiple prothombotic diseases are usually identified, mainly myeloproliferative neoplasms. New noninvasive diagnostic tools facilitate the diagnosis of these causes which may be difficult to detect in the presence of portal hypertension (PH) or liver failure.

The spectrum of symptoms ranges from an absence of signs to fulminant hepatic failure. Specific and sensitive criteria for the diagnosis of BCS have been described with Doppler ultrasound or contrast imaging (CT or MRI) so that liver biopsy is not necessary in most situations.

A minimally invasive therapeutic strategy is currently proposed for BCS patients in the West. The first step includes anticoagulant therapy and treatment of the identified cause when appropriate, to avoid the recurrence or extension of thrombosis, and nonspecific management of the complications of PH. The second step includes an attempt to recanalize and reestablish physiological venous outflow, with angioplasty/stenting. When symptoms are not resolved by this approach, the disease is managed by TIPS. If TIPS fail, or in patients with HCC, liver transplantation is performed. Long-term management must focus on close follow-up of the cause and nodules to prevent recurrent thrombosis, the complications of treatment and a misdiagnosis of HCC.

Keywords

Anticoagulation Thrombolysis Antibiotics 

Abbreviations

APLS

Antiphospholipid syndrome

BCS

BUDD Chiari Syndrome

BMB

Bone marrow biopsy

BMI

Body mass index

HCC

Hepatocellular carcinoma

HV

Hepatic vein

IVC

Inferior vena cava

MPN

Myeloproliferative neoplasm

OLT

Orthotopic liver transplantation

OP

Oestro-progestative

PH

Portal hypertension

PMI

Per million inhabitants

PNH

Paroxysomal noctural haemoglobinuria

TIPS

Transjugular porto systemic shunt

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Aurélie Plessier
    • 1
  • Audrey Payancé
    • 1
  • Dominique Valla
    • 1
  1. 1.DHU UnityPole des Maladies de l’Appareil Digestif, Service d’Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, HUPNVS, APHPClichyFrance

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