Budd-Chiari syndrome
From Medical-Wiki
Budd-Chiari syndrome (BCS) is the obstruction of venous outflow from the liver. This obstruction may be thrombotic or non-thrombotic. There is a resulting hepatic congestion as blood flows into the liver via the portal vein but then faces backpressure through the hepatic vein. This congestion leads to secondary microvascular ischemia, portal hypertension, and eventually hepatic dysfunction.
While this is a relatively rare condition, it is the major posthepatic cause of portal hypertension. In general, the most common etiology is a clotting abnormality although a third of BCS cases are idiopathic. [1]
Estimations of Budd-Chiari syndrome mortality rates vary considerably. It is fair to say, however, that they are improving. Twenty years ago, five-year survival rates were in the 50% range. They are now somewhere around 75%. [2]
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Clinical presentation of Budd-Chiari syndrome
Comorbidity
The hematologic abnormalities associated with BCS include polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria, myeloproliferative disorders, antiphospholipid antibody syndrome, and essential thrombocytosis. Inherited clotting abnormalities include protein C deficiency, protein S deficiency, antithrombin III deficiency, factor V Leiden deficiency.
Chronic infections associated with Budd-Chiari syndrome include hydatid cysts, aspergillosis, amebic abscess, syphilis, and tuberculosis.
Inflammatory diseases include Behçet disease, inflammatory bowel disease, sarcoidosis, systemic lupus erythematosus, Sjögren syndrome, and mixed connective-tissue disease. Various tumors including hepatocellular carcinoma, renal cell carcinoma, leimyosacrcoma, adrenal carcinoma, Wilms tumor, and right atrial myxoma are also associated with it. There are also a number of miscellaneous entities including alpha1-antitrypsin deficiency, trauma, pregnancy and postpartum states, membranous webs, oral contraceptives, and chronic infections.
Acute presentation
An acute presentation of BCS is accompanied by a rapid onset of abdominal pain, ascites, jaundice, hepatomegaly, and renal failure. The most common presentation is more chronic and is characterized by increasing ascites as a sign of portal hypertension. There may also be renal failure.
Fulminant form of BCS
There is also a fulminant form of the syndrome that is associated by rapidly worsening ascites, a tender liver from acute hepatomegaly, jaundice and renal failure. Left undiagnosed, this is a fatal condition. Physical findings may include icterus, ascites, hepatomegaly, splenomegaly, ankle edema, stasis ulcerations, and prominence of collateral veins. [3]
Classification of Budd-Chiari syndrome
Previously, Budd-Chiari syndrome was classified on a functional basis. This classification has been replaced by one according to the site of the venous obstruction and the presence or absence of portal vein thrombosis (PVT): hepatic vein obstruction or thrombosis without inferior vena cava (IVC) obstruction or compression; hepatic vein obstruction or thrombosis with IVC obstruction (as a result of compensatory caudate lobe hypertrophy) or thrombosis; isolated hepatic webs; and isolated IVC webs.
Radiographic findings
It is crucial to evaluate the possibility of IVC and portal-vein thrombosis as well as measure infrahepatic and suprahepatic pressures to plan treatment options. In this regard, color and pulsed-Doppler ultrasound should be the initial investigations, carrying a diagnostic sensitivity of roughly 80%. Three-dimensional MR angiography has replaced hepatic venography as the most accurate diagnostic tool. However, venography is still needed as a means od assessing the extent of thrombus propogation and measuring pressures. [4]
Treatment for Budd-Chiari syndrome
As with pulmonary vein thrombosis, all patients should be anticoagulated, first with heparin, then with coumadin.
Fulminant BCS is probably best treated with liver transplantion because of the high degree of associated tissue necrosis.
Angioplasty, portosystemic shunting, transjugular intrahepatic portosystemic shunting, and surgical shunts are also used in specific circumstances with varying degrees of success. [5]
Keywords: BUDD-Chiari Syndrome, (BCS), thrombotic hepatic vein obstruction, nonthrombotic hepatic vein obstruction, non-thrombotic hepatic vein obstruction, hepatic vein obstruction, thrombotic diathesis, congestive hepatopathy, hepatic congestion, liver congestion, membranous webs, fulminant liver disease, liver failure
