Budd-Chiari Syndrome: Illustrated Review of Current Management

John D. Horton; Francisco L. San Miguel; Jorge A. Ortiz

Disclosures

Liver International. 2008;28(4):455-466. 

In This Article

Presentation

The majority of patients with BCS present with hepatomegaly, right upper quadrant pain and abdominal ascites.[5,14] Lower extremity oedema is another common finding on physical exam. Some patients may feature signs of portal hypertension such as variceal bleeding and refractory ascites without a significant impact on liver function. A high serum-ascites gradient (≥1.1g/dl) is usually found.[1]

Young children (<10 years of age) account for 1-7% of all cases of BCS. This group often presents late in the course of disease, by which time irreversible pathology may be present.[15]

The most common underlying disorders in patients with BCS are myeloproliferative disorders such as polycythaemia vera and essential thrombocytosis.[1] The prevalence of myeloproliferative disorders in this population can be as high as 53%. In Western nations, factor V Leiden (25%) and factor II gene mutation (5%) are other common aetiologies. Some patients may have a combination of thrombophilias.[5] Other common associations include oral contraceptive use, protein C and S deficiencies, antiphospholipid syndrome, antithrombin III deficiency, paroxysmal nocturnal haemoglobinuria, cancer, Bechet's syndrome and trauma.[16] BCS after pregnancy has been described and is associated with a particularly poor prognosis.[17] Twenty per cent of cases of BCS are idiopathic.[1]

The Budd-Chiari syndrome is termed primary or secondary based on the aetiology and site of venous outflow obstruction. Primary BCS originates from within the lumen of the veins or venules and results from thrombus, webs or endophlebitis. Secondary BCS results from an extra-luminal lesion such as tumour, abscess or cyst, which can invade the lumen or cause extrinsic compression of the hepatic venous outflow tract.[4]

Patients with BCS present with varying degrees of symptomatology and acuity. The clinical presentation of patients with BCS is governed by the extent and swiftness of hepatic outflow obstruction juxtaposed to the body's ability to decompress the liver via development of collateral blood flow. Based on this pathophysiology, patients can be divided into the following categories: fulminant, acute, subacute or chronic.[13] The subacute form is the most common presentation and is characterized by slow accumulation of ascites. Hepatocellular damage is minimal due to the development of hepatic and portal venous collaterals. The fulminant form is rare and is defined as the development of hepatic encephalopathy within 8 weeks of the onset of jaundice. Acute BCS has a rapid onset, intractable ascites and a short duration of symptoms. Collateral venous channels do not develop in the acute form of BCS. Chronic BCS usually occurs in the background of cirrhosis. A report by Dilawari included 177 patients with BCS and used a variation of the above classification scheme. This patient population presented with the following distribution: 7% acute fulminant, 28% acute and 65% chronic.[18]

Presentation of BCS has been classified using a variety of schemes. Langlet's recent clinicopathological classification demonstrated prognostic value. This scheme divides patients into the following types: type I (acute injury only, corresponding to onset of hepatic outflow obstruction), type II (chronic lesions only, corresponding to the sequelae of remote hepatic outflow obstruction) and type III (acute injury superimposed on chronic lesions). This patient population had the following distribution: 7% type I, 45% type II and 48% type III. Patients with type I BCS had the best prognosis whereas those with type III had the worst.[19]

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