(See also Overview of Vascular Disorders of the Liver Overview of Vascular Disorders of the Liver The liver has a dual blood supply. The portal vein (which is rich in nutrients and relatively high in oxygen) provides two thirds of blood flow to the liver. The hepatic artery (which is oxygen-rich)... read more .)
Etiology of Budd-Chiari Syndrome
In the Western world, the most common cause is a clot obstructing the hepatic veins and the adjacent inferior vena cava. Clots commonly result from the following:
Thrombotic conditions (eg, protein C or S deficiency, antiphospholipid syndrome, antithrombin III deficiency, factor V Leiden mutation, pregnancy, oral contraceptive use)
Hematologic disorders (eg, myeloproliferative disorders Overview of Myeloproliferative Neoplasms Myeloproliferative neoplasms are clonal proliferations of bone marrow stem cells, which can manifest as an increased number of platelets, red blood cells (RBCs), or white blood cells (WBCs)... read more such as polycythemia Polycythemia Vera Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by an increase in morphologically normal red cells (its hallmark), but also white cells and platelets. Ten to 30%... read more and paroxysmal nocturnal hemoglobinopathy)
Connective tissue disorders
Infection (eg, hydatid cyst, amebiasis)
Tumor invasion of the hepatic vein (eg, hepatocellular carcinoma Hepatocellular Carcinoma Hepatocellular carcinoma usually occurs in patients with cirrhosis and is common in areas where infection with hepatitis B and C viruses is prevalent. Symptoms and signs are usually nonspecific... read more or renal cell carcinoma Renal Cell Carcinoma Renal cell carcinoma (RCC) is the most common renal cancer. Symptoms can include hematuria, flank pain, a palpable mass, and fever of unknown origin (FUO). However, symptoms are often absent... read more )
Sometimes Budd-Chiari syndrome begins during pregnancy and unmasks a previously asymptomatic hypercoagulability disorder.
The cause of obstruction is often unknown. In Asia and South Africa, the basic defect is often a membranous obstruction (webs) of the inferior vena cava above the liver, likely representing recanalization of a prior thrombus in adults or a developmental flaw (eg, venous stenosis) in children. This type of obstruction is called obliterative hepatocavopathy.
Budd-Chiari syndrome usually develops over weeks or months. When it does develop over a period of time, cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic fibrosis that has resulted in widespread distortion of normal hepatic architecture. Cirrhosis is characterized by regenerative nodules surrounded by dense... read more and portal hypertension Portal Hypertension Portal hypertension is elevated pressure in the portal vein. It is caused most often by cirrhosis (in developed countries), schistosomiasis (in endemic areas), or hepatic vascular abnormalities... read more tend to develop.
Symptoms and Signs of Budd-Chiari Syndrome
Manifestations range from none (asymptomatic) to fulminant liver failure Acute Liver Failure Acute liver failure is caused most often by drugs and hepatitis viruses. Cardinal manifestations are jaundice, coagulopathy, and encephalopathy. Diagnosis is clinical. Treatment is mainly supportive... read more or cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic fibrosis that has resulted in widespread distortion of normal hepatic architecture. Cirrhosis is characterized by regenerative nodules surrounded by dense... read more . Symptoms vary depending on whether the obstruction occurs acutely or over time.
Acute obstruction (in about 20%) causes fatigue, right upper quadrant pain, nausea, vomiting, mild jaundice, tender hepatomegaly, and ascites. It typically occurs during pregnancy. Fulminant liver failure with encephalopathy is rare. Aminotransferase levels are quite high.
Chronic outflow obstruction (developing over months) may cause few or no symptoms until it progresses, or it may cause fatigue, abdominal pain, and hepatomegaly. Lower-extremity edema and ascites Ascites Ascites is free fluid in the peritoneal cavity. The most common cause is portal hypertension. Symptoms usually result from abdominal distention. Diagnosis is based on physical examination and... read more may result from venous obstruction, even in the absence of cirrhosis. Cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic fibrosis that has resulted in widespread distortion of normal hepatic architecture. Cirrhosis is characterized by regenerative nodules surrounded by dense... read more may develop as well as manifestations of portal hypertension Portal Hypertension Portal hypertension is elevated pressure in the portal vein. It is caused most often by cirrhosis (in developed countries), schistosomiasis (in endemic areas), or hepatic vascular abnormalities... read more , including variceal bleeding Varices Varices are dilated veins in the distal esophagus or proximal stomach caused by elevated pressure in the portal venous system, typically from cirrhosis. They may bleed massively but cause no... read more , massive ascites, splenomegaly, hepatopulmonary syndrome Hepatopulmonary Syndrome Hepatopulmonary syndrome is hypoxemia caused by pulmonary microvascular vasodilation in patients with portal hypertension; dyspnea and hypoxemia are worse when the patient is upright. Hepatopulmonary... read more , or a combination. Complete obstruction of the inferior vena cava causes edema of the abdominal wall and legs plus visibly tortuous superficial abdominal veins from the pelvis to the costal margin.
Diagnosis of Budd-Chiari Syndrome
Clinical evaluation and liver tests
Budd-Chiari syndrome is suspected in patients with hepatomegaly, ascites, liver failure, or cirrhosis when there is no obvious cause (eg, alcohol abuse, hepatitis) or when the cause is unexplained.
Liver tests Laboratory Tests of the Liver and Gallbladder Laboratory tests are generally effective for the following: Detecting hepatic dysfunction Assessing the severity of liver injury Monitoring the course of liver diseases and the response to treatment... read more are usually abnormal; the pattern is variable and nonspecific. The presence of risk factors for thrombosis increases the consideration of this diagnosis.
Imaging Imaging Tests of the Liver and Gallbladder Imaging is essential for accurately diagnosing biliary tract disorders and is important for detecting focal liver lesions (eg, abscess, tumor). It is limited in detecting and diagnosing diffuse... read more usually begins with abdominal Doppler ultrasonography, which can show the direction of blood flow and the site of obstruction. Magnetic resonance angiography and CT are useful if ultrasonography is not diagnostic. Conventional angiography (venography with pressure measurements and arteriography) is necessary if therapeutic or surgical intervention is planned.
Liver biopsy Liver Biopsy Liver biopsy provides histologic information about liver structure and evidence of liver injury (type and degree, any fibrosis); this information can be essential not only to diagnosis but also... read more is done occasionally to diagnose the acute stages and determine whether cirrhosis has developed.
Prognosis for Budd-Chiari Syndrome
Without treatment, most patients with complete venous obstruction die of liver failure within 3 to 5 years. For patients with incomplete obstruction, the course varies.
Treatment of Budd-Chiari Syndrome
Restoration and maintenance of adequate venous outflow
Treatment varies according to onset (acute vs chronic) and severity (fulminant liver failure vs decompensated cirrhosis or stable/asymptomatic). The cornerstones of management are
Giving supportive therapy directed at complications (eg, ascites Ascites Ascites is free fluid in the peritoneal cavity. The most common cause is portal hypertension. Symptoms usually result from abdominal distention. Diagnosis is based on physical examination and... read more , liver failure Acute Liver Failure Acute liver failure is caused most often by drugs and hepatitis viruses. Cardinal manifestations are jaundice, coagulopathy, and encephalopathy. Diagnosis is clinical. Treatment is mainly supportive... read more , esophageal varices Varices Varices are dilated veins in the distal esophagus or proximal stomach caused by elevated pressure in the portal venous system, typically from cirrhosis. They may bleed massively but cause no... read more )
Decompressing the congested liver (ie, maintaining venous outflow)
Preventing propagation of the clot
Aggressive interventions (eg, thrombolysis, stents) are used when the disease is acute (eg, within 4 weeks and in the absence of cirrhosis). Thrombolysis can dissolve acute clots, allowing recanalization and so relieving hepatic congestion. Radiologic procedures, such as angioplasty, stenting, and/or portosytemic shunts, can have a major role.
For caval webs or hepatic venous stenosis, decompression via percutaneous transluminal balloon angioplasty with intraluminal stents can maintain hepatic outflow. When dilation of a hepatic outflow narrowing is not technically feasible, transjugular intrahepatic portosystemic shunting (TIPS) and various surgical shunts can provide decompression by diverting blood flow into the systemic circulation. Portosystemic shunts are typically not used if hepatic encephalopathy Portosystemic Encephalopathy Portosystemic encephalopathy is a neuropsychiatric syndrome that can develop in patients with liver disease. It most often results from high gut protein or acute metabolic stress (eg, gastrointestinal... read more is present; such shunts may worsen liver function. Further, clots may form in shunts, especially if patients have a hematologic or thrombotic disorder.
Long-term anticoagulation is often necessary to prevent recurrence. Liver transplantation Liver Transplantation Liver transplantation is the 2nd most common type of solid organ transplantation. (See also Overview of Transplantation.) Indications for liver transplantation include Cirrhosis (70% of transplantations... read more may be lifesaving in patients with fulminant disease or decompensated cirrhosis.
The most common cause of Budd-Chiari syndrome (obstruction of hepatic venous outflow) is a clot blocking the hepatic veins and inferior vena cava.
Consider the diagnosis if patients have typical findings (eg, hepatomegaly, ascites, liver failure, cirrhosis) that are unexplained or if they have abnormal liver test results and risk factors for thrombosis.
Confirm the diagnosis using Doppler ultrasonography or, if results are inconclusive, magnetic resonance angiography or CT, or venography.
Restore venous outflow (eg, with thrombolysis, angioplasty, stents), and treat complications.
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