VIDEO TC :
Contrast enhancement axial CT scan showing a large ascites, and thrombosis hepatic veins.
VIDEO US:
Important echogenic alteration of the liver.
Non visualization of the hepatic veins.
With Color and Power Doppler sonography , absence flow in the hepatic veins.
Flow in the portal vein and inferior vena cava
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CLINICAL PRESENTATION
PRESENTATION
A 27-years-old woman was admitted with a 5 days history of diarrhoea, nausea, vomiting, abdominal pain and with a 2 days history of abdominal distension and jaundice. She had not relevant past medical history except for use of oral contraceptive pills.
Her mother had a history of deep vein thrombosis.
On clinical examination at admission the patient looked well, apart from severe jaundice and abdominal distension for ascites, with normal mental state. In the next twenty-four hours she deteriorated and became confused with encefalopathy grade II-III.
Laboratory investigations showed a picture of severe acute liver failure with disseminated intravascular coagulopathy (DIC).
Coagulopathy was treated with fresh frozen plasma infusion plus thrombolysis with LMW heparin subcutaneous. At the same time, because of encephalopathy and coagulopathy resistant to vitamin K infusion, she was listed for urgent liver transplantation.
FINAL DIAGNOSIS:
Budd Chiari Syndrome
DISCUSSION
Budd Chiari Syndrome is a condition characterised by obstruction of hepatic venous outflow, anywhere from small hepatic venules to the inferior caval vein and the right atrium, but mainly in main hepatic veins.
Etiology: several disorders can cause thrombosis or obstruction of hepatic veins. In order of frequency: myeloproliferative disorders; hypercoagulative state (with oral contraceptive as addictive factor); tumours, infections, hydiopatic.
Clinical features: typically are abdominal pain, ascites and hepatomegaly. Depending on the rapidity of vein obstruction it is possible to have acute-fulminant liver failure or chronic liver dysfunction with jaundice and refractory ascites even on adequate diuretic treatment.
Diagnosis: ultrasound with color Doppler of the liver; CT Scan.
Treatment: in case of rapid deterioration of liver function with acute or fulminant liver failure (with coagulopathy and encephalopathy) the only choice is urgent liver transplantation. In chronic clinical forms medical treatment with anticoagulation therapy, diuretics and paracentesis on demand can give some results. In case of good liver function and ascites as the only manifestation of portal hypertension, venous decompression with TIPPS or other porto-caval shunts can be considered. In case of portal hypertensione and chronic liver dysfunction liver transplantation is the best choice even if most patients require long life anticoagulation therapy.
REFFERENCES :
Brancatelli G, Vilgrain V, Federle MP, Hakime A, Lagalla R, Iannaccone R. Budd-Chiari syndrome: spectrum of imaging findings. AJR Am J Roentgenol. Feb 2007;188(2):W168-76.
Chawla Y, Kumar S, Dhiman RK, et al. Duplex Doppler sonography in patients with Budd-Chiari syndrome. J Gastroenterol Hepatol. Sep 1999;14(9):904-7.
McKusick MA. Imaging findings in Budd-Chiari syndrome. Liver Transpl. Aug 2001;7(8):743-4.
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