Budd-Chiari syndrome causes serious problems in liver blood drainage. It is generally characterized by obstruction of the hepatic veins, leading to increased pressure in the liver. As a result, liver enlargement and functional disorders occur. Portal hypertension and ascites may also develop as additional complications. Pressure caused by both primary thrombosis and tumors are the main causes of this syndrome. Treatment methods vary depending on disease severity and often require anticoagulation or more radical therapies.
| Medical Name | Budd-Chiari Syndrome |
| Affected Areas | Liver (obstruction in hepatic veins) |
| Causes | Thrombosis in hepatic veins, clotting disorders, cancer, pregnancy, congenital anomalies |
| Symptoms | Abdominal pain (especially in the right upper quadrant), abdominal swelling (ascites), liver enlargement (hepatomegaly), jaundice, leg swelling, weight loss |
| Diagnostic Methods | Doppler ultrasound, magnetic resonance imaging (MRI), computed tomography (CT), liver biopsy, venography |
| Treatment Methods | Anticoagulant drugs, thrombolytic therapy, stent placement, shunt surgery, TIPS when vessels cannot be opened, liver transplantation (in advanced cases) |
| Possible Complications | Liver failure, portal hypertension, renal failure, liver cirrhosis |
| Prevention Methods | Appropriate management of patients with clotting disorders, risk factor control |
| Recovery Time | Varies with treatment; mild cases can be controlled with therapy, while severe cases may require liver transplantation |
Interventional Radiology / Interventional Neuroradiology
Prof. Dr. Özgür KILIÇKESMEZ
What Is Budd-Chiari Syndrome?
Budd-Chiari syndrome is a rare condition characterized by obstruction of the hepatic veins. The cause of the obstruction can vary widely and is classified into two main types: primary and secondary. In the primary type, the veins are usually blocked by intrinsic causes such as thrombosis or phlebitis. In the secondary type, the veins are typically compressed externally by a lesion, most often tumors. Both situations impede blood flow in the liver, leading to various health issues. Diagnosis and treatment depend on the location and cause of the obstruction.
What Are the Causes of Budd-Chiari Syndrome?
Budd-Chiari syndrome is a serious health problem resulting from obstruction of hepatic venous circulation. Underlying causes are generally associated with hypercoagulable states. The different causes include:
Myeloproliferative Disorders:
- Polycythemia vera
- Essential thrombocythemia
These disorders lead to faster-than-normal blood clotting and can cause Budd-Chiari syndrome, observed in more than half of cases.
Malignancies:
- Liver cancer
- Adrenal gland cancer
- Renal cell carcinoma
- Leiomyosarcoma
- Right atrial myxoma
- Wilms tumor
These malignancies can directly compress or invade the veins, resulting in venous obstruction. About one in ten cases is due to these causes.
Liver Lesions:
- Hepatic cysts
- Adenomas
- Cystadenomas
- Invasive aspergillosis
- Aortic aneurysm
Such lesions in the liver can compress the veins, leading to Budd-Chiari syndrome.
Pregnancy and Oral Contraceptive Use: Pregnancy and oral contraceptives can increase clotting and account for about twenty percent of cases.
Idiopathic Causes: Twenty percent of cases occur without an identifiable cause.
Other hypercoagulable conditions include:
- Factor V (Leiden) mutation
- Antiphospholipid antibody syndrome
- Antithrombin deficiency
- Protein C deficiency
- Paroxysmal nocturnal hemoglobinuria
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How Common Is Budd-Chiari Syndrome?
Budd-Chiari syndrome is more frequently diagnosed in the third or fourth decade of life and is usually seen in female patients outside of Asia. In Asia, however, it is more commonly reported in men. It often involves obstruction of the inferior vena cava alone or together with hepatic veins. These differences may be due to genetic or environmental factors. Symptoms include liver enlargement, pain, and leg swelling. Treatment varies according to the severity and location of the obstruction.
What Happens During Budd-Chiari Syndrome?
During Budd-Chiari syndrome, significant changes occur in the liver. Obstruction of two hepatic veins stretches the liver capsule, causing severe pain. As a result, sinusoids dilate and interstitial fluid filtration increases. The filtered fluid exceeds lymphatic capacity and leads to ascites. This process involves:
- Venous obstruction stretches the liver capsule, causing pain.
- Sinusoidal dilation increases interstitial fluid filtration.
- Filtered fluid overwhelms lymphatic drainage, leading to ascites.
Thrombosis and impaired venous flow cause portal hypertension and reduce liver perfusion. This leads to hypoxic damage in centrilobular hepatocytes, causing acute liver failure in acute cases and ascites and hepatomegaly in chronic cases despite preserved liver function. Over time, this process can lead to fibrosis and eventually cirrhosis. The caudate lobe, which drains directly into the inferior vena cava, is most affected and hypertrophies when obstructed.
Microscopic Examination of Budd-Chiari Syndrome
On liver biopsy, Budd-Chiari syndrome shows characteristic features. Centrilobular hepatocyte necrosis is observed due to impaired blood flow. As the disease progresses, sinusoidal dilation becomes prominent, directly related to halted blood flow.
In some cases, macronodules may be present in the liver tissue, indicating disease severity and liver impact. These microscopic findings provide critical information for diagnosis and treatment, helping to assess disease progression.
What Are the Symptoms of Budd-Chiari Syndrome?
Budd-Chiari syndrome results from hepatic vein obstruction and presents with various symptoms. Patients may experience symptoms in acute, subacute, and chronic phases.
In the acute phase, symptoms develop rapidly over weeks, with sudden abdominal pain, jaundice, and ascites. Hepatic encephalopathy and renal failure may also occur. Venous collaterals are usually absent at this stage.
In the subacute phase, symptoms may last up to three months. Patients gradually develop mild ascites and hepatic necrosis. Venous collaterals may form, allowing alternative blood flow pathways.
In the chronic phase, symptoms appear over a longer period. Patients often present with cirrhosis complications and venous collaterals. Half of patients in this phase may develop renal failure.
Symptom list:
- Sudden-onset ascites
- Painful hepatomegaly
- Jaundice
- Ascites
- Hepatomegaly
- Splenomegaly
- Pedal edema
- Stasis ulcers
How Is Budd-Chiari Syndrome Diagnosed?
Diagnosing Budd-Chiari syndrome is challenging. Various tests play key roles. First, Doppler ultrasound is used; it detects thrombi and vascular anomalies with 85–90% sensitivity and specificity. If ultrasound is inconclusive, CT or MRI is performed. Both methods have high sensitivity and specificity, with MRI preferred due to its noninvasive nature.
Ultrasound findings include:
- Webs and thrombi in the inferior vena cava and hepatic veins
- Reduced IVC diameter
- Hepatic vein thrombosis
- Enlarged caudate lobe
- Presence of ascites
- Intrahepatic or extrahepatic collaterals
- Monophasic flow in hepatic veins
- High flow velocities at stenotic segments in IVC or hepatic veins
If diagnosis remains uncertain after radiologic evaluation, invasive venography is recommended to locate and assess the severity of the obstruction. Diagnostic paracentesis may also be performed to analyze ascitic fluid, showing high protein levels and low white blood cell counts.
How Is Budd-Chiari Syndrome Treated?
Various treatments exist for Budd-Chiari syndrome. Initially, balloon angioplasty is performed to open short-segment stenoses in hepatic veins or the inferior vena cava. This reduces portal hypertension and improves liver function in localized obstructions. If angioplasty is insufficient, endovascular stent placement is used to maintain venous patency, providing long-term success in complex obstructions.
- Balloon Angioplasty: Effective for opening localized obstructions.
- Endovascular Stent Placement: Ensures long-term patency in complex cases.
If these methods fail, Transjugular Intrahepatic Portosystemic Shunt (TIPS) can be applied in advanced cases with significant liver damage. TIPS is valuable for refractory ascites or variceal bleeding and serves as a bridge to liver transplantation.
What Other Conditions Should Be Considered?
When diagnosing Budd-Chiari syndrome, other potential conditions must be considered. Right and congestive heart failure can affect the liver and cause confusion. Metastatic and alcoholic liver diseases show similar symptoms. Granulomatous diseases and alpha-1 antitrypsin deficiency should also be evaluated. Infectious and drug-induced hepatitis, Fitz-Hugh–Curtis syndrome, Niemann–Pick disease type C, neonatal hemochromatosis, biliary atresia, congenital hepatic fibrosis, cystic fibrosis, metabolic disorders, and hemochromatosis are among the rare conditions to consider.
Prognosis of Budd-Chiari Syndrome
Prognosis varies by factors such as age, Child-Pugh score, and renal function. Younger patients and those with low Child-Pugh scores generally have better outcomes. Low serum creatinine and absence of ascites predict slower disease progression. Involvement of all hepatic veins and/or the portal vein, presence of ascites, and older age are associated with poorer prognosis. High Child-Pugh scores and chronic comorbidities increase risk and influence treatment and follow-up strategies.
What Are the Complications of Budd-Chiari Syndrome?
Untreated Budd-Chiari syndrome can lead to serious complications. Hepatic encephalopathy may develop due to impaired detoxification. Variceal bleeding is common from weakened vessels under high portal pressure. Hepatorenal syndrome, a form of renal failure, arises as a chronic feature of portal hypertension. Ascites increases the risk of bacterial peritonitis. Finally, there is an elevated risk of hepatocellular carcinoma in these patients.
Prof. Dr. Özgür Kılıçkesmez graduated from Cerrahpaşa Medical Faculty in 1997. He completed his specialization at Istanbul Education and Research Hospital. He received training in interventional radiology and oncology in London. He founded the interventional radiology department at Istanbul Çam and Sakura City Hospital and became a professor in 2020. He holds many international awards and certificates, has over 150 scientific publications, and has been cited more than 1500 times. He is currently working at Medicana Ataköy Hospital.
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