PubMed · 2862472
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1985-08-10. Hepatic outflow obstruction.. https://pubmed.ncbi.nlm.nih.gov/2862472/
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Budd-Chiari syndrome occurs when venous outflow from the liver is obstructed. The obstruction may occur at any point from the hepatic venules to the left atrium. The syndrome most often occurs in patients with underlying thrombotic disorders such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria and pregnancy. It may also occur secondary to a variety of tumours, chronic inflammatory diseases and infections. Imaging plays an important role both in establishing the diagnosis of Budd-Chiari syndrome as well as evaluating for underlying causes and complications such as portal hypertension. In this review article, we discuss the role of modern imaging in the evaluation of Budd-Chiari syndrome.
BACKGROUND: Hepatic venous outflow obstruction (HVOO) is a rare disorder that occurs predominantly due to a hypercoagulable state. The syndrome may result from hepatic vein obstruction, inferior vena cava obstruction or a combination of both and manifests with post-sinusoidal portal hypertension. The presentation may be fulminant with poor prognosis or as either acute, subacute or chronic forms with relatively better prognosis. Portosystemic shunt surgery (PSS) has thus far been the mainstay of treatment for HVOO. However, over the last decade, transjugular intrahepatic portosystemic 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 investigations and treatment was performed using Medline and additional library searches. RESULTS: Portosystemic shunts form the mainstay of treatment for patients with subacute presentation (preserved liver function) with medically intractable ascites or recurrent variceal hemorrhage. Excellent results with 5-year survival of more than 90% have been 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 transplantation 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.
Budd-Chiari syndrome is an uncommon, often fatal disorder resulting from an obstructed hepatic venous outflow tract. The obstructive lesion is situated in the main hepatic veins, in the inferior vena cava or in both. The nature, location and extension of the obstruction can be displayed on diagnostic imaging techniques. In addition to this direct evidence, the indirect findings of venous obstruction such as the presence of intra- and extrahepatic collateral veins, when combined with the altered morphology and enhancement pattern of the liver enables one to arrive at a confident diagnosis. In patients with suspected Budd-Chiari syndrome, gray-scale sonography with complementary support of color and pulsed Doppler examinations is the first step in approaching the diagnosis. It is followed by a contrast-enhanced cross-sectional technique, preferrentially by MR angiography. The patients with a high clinical suspicion of Budd-Chiari syndrome may undergo hepatic venography or venacavography directly so that a potential of recanalization (e.g. percutaneous transluminal angioplasty with or without stent placement or TIPS) of the obstructed segment under the guidance of these techniques would not be delayed.