[MIM 600 880]

Very rare: 1/50,000 to 1/100,000. Obstruction of the venous drainage of the liver at the level of centrilobular veins, hepatic veins or the terminal segment of the inferior vena cava. The increased sinusoidal pressure in the liver leads to liver dysfunction, hepatosplenomegaly and portal hypertension with ascites. However, 15 to 20% of cases are asymptomatic if there are large  collateral veins that circumvent the obstacle.If left untreated, this process may lead to a progressive destruction of hepatocytes (clinical picture of fulminant hepatitis), rapidly progressing liver failure (acute form) or cirrhosis (chronic form).

Two forms:

• primary Budd-Chiari syndrome: due to intraluminal obstruction linked to:

-         a congenital thrombophilia: deficiency in protein C, in protein S  or in antithrombin III, mutation in the factor V of Leyden (heterozygote), mutation G20210A of prothrombin, hyperhomocysteinemia, hypereosinophilic syndrome, mutation of factor II, paroxysmal nocturnal hemoglobinuria 

-         an acquired thrombophilia : myeloproliferative syndrome with JAK2 mutation, antiphospholipids syndrome, Behcet's disease, oral contraception, inflammatory disease of the digestive tract (some cases of celiac disease), pregnancy

-         an intravascular membrane at the level of the inferior vena cava  or Chiari veins

• secondary Budd-Chiari syndrome: due to a parasitic disease or a tumor that compresses the liver.

Treatment: individualized and based on the clinical presentation and liver function.

-        in situ thrombolysis with or without endovascular recanalization by interventional radiology

-        intra- or extrahepatic portosystemic shunt

-        liver transplantation with thrombectomy

Anesthetic implications: 

poor liver function, portal hypertension and ascites; risk of hepato-pulmonary syndrome (see this term) or hepato-renal syndrome.

References : 

• Cauchi JA, Oliff S, Baumann U, Mirza D et al. 
  The Budd-Chiari syndrome in children : the spectrum of management. 
  J Pediatr Surg 2006; 41: 1919-23.
• Plessier A, Valla D-C. 
  Budd-Chiari syndrome. 
  Semin Liver Dis 20008 ; 28 : 259-69.
• Jones TL, O’Beirne J, Goode A, Harrison S. 
  Anaesthesia for caesarian section in a patient with Budd-Chiari syndrome and hepatopulmonary syndrome post liver transplantation. 
  Int J Obster Anesth 2011; 20: 169-73.
• Boskovic A, Kitic I, Stankovic I, Prokic D, Zlatar N. 
  Primary Budd-Chiari syndrome in a 3-year-old boy with homozygous factor V Leyden G1691A mutation. 
  Eur J Pediatr 2014; 173: 393-5.

Updated: November 2019