Budd-Chiari syndrome
Budd-Chiari Syndrome is a rare liver pathology caused by partial or complete blockage (occlusion) of blood outflow through the hepatic veins. Due to thrombosis, blood cannot normally leave the liver and enter the inferior vena cava. This leads to acute or chronic blood stasis in the organ, a sharp increase in pressure in the portal vein system (portal hypertension), oxygen starvation of hepatocytes (ischemia), and their subsequent death with the development of cirrhosis.
Causes of Budd-Chiari Syndrome
The main cause of Budd-Chiari syndrome is the body's predisposition to increased blood clotting (hypercoagulation) and clot formation. Conditions that provoke hepatic vein thrombosis include:
- Myeloproliferative blood disorders (polycythemia vera, essential thrombocythemia);
- Hypercoagulation disorders: These include protein C or S deficiency, antiphospholipid syndrome, antithrombin III deficiency, Factor V Leiden mutation, and systemic lupus erythematosus.
- Use of oral contraceptives (especially against the background of a genetic predisposition to thrombosis);
- Infectious processes or abdominal injuries.
- Formations in the liver or kidneys: Malignant or benign formations that may compress the hepatic veins or the inferior vena cava.
- Sickle cell anemia: a blood disease in which red blood cells change shape from round to sickle-shaped.
- Inflammatory bowel diseases (IBD): a group of diseases that cause inflammation in the digestive system.
- Pregnancy: pregnancy increases the risk of blood clots. This is especially true if there is an existing condition that promotes clot formation.
Every fifth case of Budd-Chiari syndrome is idiopathic. This means that medical professionals cannot determine a clear cause of the condition.
Complications
- Portal hypertension: increased pressure in the portal vein. This vein carries blood from the digestive tract and spleen to the liver.
- Varicose veins: enlarged veins in the esophagus, stomach, or rectum.
- Ascites: accumulation of fluid in the abdominal cavity.
- Cirrhosis: the end stage of chronic liver disease
- Acute (fulminant) liver failure.
Types of Budd-Chiari Syndrome
Doctors classify Budd-Chiari syndrome based on the duration of its onset and the severity of symptoms.
- Acute Budd-Chiari syndrome with acute liver failure: symptoms develop rapidly (within a few days or weeks), and liver function declines quickly.
- Acute Budd-Chiari syndrome without liver failure: symptoms develop quickly, but without loss of liver function.
- Subacute Budd-Chiari syndrome: symptoms appear more slowly, usually over several months. This is the most common type of Budd-Chiari syndrome.
- Chronic Budd-Chiari syndrome: symptoms are usually not noticeable until the blockage causes irreversible scarring (cirrhosis) of the liver.
Budd-Chiari syndrome can be primary or secondary:
- Primary Budd-Chiari syndrome occurs due to the narrowing or blockage of blood vessels. Causes include a thrombus (soft clot) or blockage by a cluster of tumor cells. Diseases that cause inflammation of the hepatic veins may also contribute.
- Secondary Budd-Chiari syndrome occurs due to a problem outside the blood vessels. Causes include a malignant (cancerous) or benign (non-cancerous) tumor pressing on one of the hepatic veins or the inferior vena cava (IVC). The IVC drains blood from the hepatic veins to the right side of the heart. Conditions that damage liver cells or compress the hepatic veins may also be a cause. These include some fungal infections, trauma, or chemotherapy.
Symptoms of Budd-Chiari Syndrome
The clinical picture depends on the speed of vascular occlusion and the number of affected veins. The classic course of the syndrome is characterized by a specific triad of symptoms:
- Sudden or gradually increasing pain in the right upper quadrant;
- Rapid enlargement of the liver (hepatomegaly), the organ becomes dense and painful upon palpation;
- Rapid accumulation of free fluid in the abdominal cavity (ascites), which is difficult to treat with diuretics.
Additionally, the following may be observed:
- jaundice
- enlarged spleen (splenomegaly)
- enlarged subcutaneous veins on the abdomen
- gastrointestinal bleeding from varicose veins of the esophagus.
In the fulminant form, acute liver failure with impaired consciousness develops quickly.
Diagnosis of Budd-Chiari Syndrome at "Oberig" Clinic
- Ultrasound examination of the liver with Doppler sonography of blood vessels and elastography (non-invasive measurement of liver stiffness to determine the degree of fibrosis) — examinations are performed on expert-class ultrasound system ACUSON Sequoia from SIEMENS.
- Computed tomography (CT) (including with contrast enhancement) — examinations are performed on a modern 128-slice CT scanner from SIEMENS.
- Magnetic resonance imaging (MRI) - the clinic is equipped with the latest Vida 3 Tesla MRI scanner and is a reference center for Siemens AG in Ukraine.
- Esophagogastroduodenoscopy — is performed to assess the presence and degree of varicose veins of the esophagus and stomach - on FUJI and Olympus equipment with artificial intelligence. "Oberig" Clinic is a leader in the implementation of the latest technologies in endoscopy.
- For histological verification of the diagnosis - ultrasound-guided biopsy performed by an expert in interventional radiology Y. E. Chirkov (as indicated).
- Laboratory tests (list according to the doctor's recommendations) in our own certified laboratory.
Treatment of Budd-Chiari Syndrome at "Oberig" Clinic
Conservative therapy includes taking anticoagulants to prevent further clot formation and symptomatic treatment of disease complications. If possible, treatment is provided for the disease or condition that led to the venous occlusion.
When irreversible changes develop in the liver, the only life-saving treatment method is liver transplantation.
Why choose "Oberig" Clinic for the treatment of Budd-Chiari Syndrome?
- National leadership in the field of living-related liver transplantation: as of 1.03.2026, 212 liver transplantations have been performed for adults and children, 205 of which were from a living related donor.
- Treatment effectiveness: comparable to the best world transplant centers, patient survival after transplantation is over 85%.
- Treatment accessibility: free transplantation for patients under the agreement with the National Health Service of Ukraine (NHSU).
- Full-cycle clinic: the entire necessary complex of diagnostic services (MRI, CT, ultrasound, laboratory diagnostics, biopsy, pathohistology) and all types of treatment (conservative therapy, hepatobiliary surgery, transplantation) are available in one clinic.
- Patient safety: ensuring infectious monitoring at all stages of treatment.
Sign up for a consultation with Oberig Clinic doctors
Online consultation appointment: https://my.oberig.ua/ua/appointment/ or call +38 044 521 30 03
