Related Experiment Video
Updated: May 20, 2026
Laparoscopic Anterior Right Hepatectomy: A Single-Center Experience
Published on: December 4, 2023
Primary Budd-Chiari syndrome - a single center experience
Joanna Raszeja-Wyszomirska1, Joanna Mieżyńska-Kurtycz, Wojciech Marlicz
1Liver Unit, Pomeranian Medical University, Szczecin, Poland. jorasz@sci.pum.edu.pl
Insights
Budd-Chiari syndrome (BCS) management requires strict adherence to guidelines. Anticoagulation, TIPS, and liver transplants (OLT) are effective treatments for this rare, potentially lethal condition.
Area of Science:
- Hepatology
- Vascular Medicine
- Thrombosis Research
Background:
- Budd-Chiari syndrome (BCS) is a rare but potentially fatal condition caused by hepatic vein thrombosis.
- Prompt diagnosis and management are critical, as BCS can lead to liver failure within three years if untreated.
Purpose of the Study:
- To retrospectively analyze the diagnostic and therapeutic strategies for Budd-Chiari syndrome.
- To evaluate the outcomes of BCS patients managed between 2004 and 2011.
Main Methods:
- Diagnosis confirmed using contrast CT-angiography and Doppler ultrasound.
- Retrospective analysis of 20 BCS patients managed over an 8-year period.
- Treatment included anticoagulation, Transjugular Intrahepatic Portosystemic Shunt (TIPS), and Orthotopic Liver Transplantation (OLT).
Main Results:
- Myeloproliferative disorders, end-stage liver disease, and protein C deficiency were common causes of BCS.
- The majority of patients received anticoagulation, with some undergoing TIPS or LTx.
- A significant proportion of patients (14/20) were alive and well at least one year post-diagnosis, indicating treatment efficacy.
Conclusions:
- Adherence to diagnostic and therapeutic guidelines is crucial for successful BCS management.
- Anticoagulation, TIPS, and OLT demonstrate efficacy in treating Budd-Chiari syndrome.
- Effective management strategies can significantly improve patient survival and outcomes.
Background/Aims:
Budd-Chiari syndrome (BCS) is recognized as a clinical manifestation of various prothrombotic conditions which may be lethal within 3 years of the onset of symptoms if untreated. This study is a retrospective analysis of patients with BCS managed between 2004 and 2011.
Methodology:
The diagnosis was confirmed with contrast CT-angiography and/or Doppler ultrasound.
Results:
BCS was diagnosed in 20 patients (11 females and 9 males), median age 38 years (ranging from 18 to 56). Twelve patients were referred as acute BCS for the liver transplant (LTx) assessment. Thrombosis of the hepatic veins was caused by myeloproliferative disorders (n=8), end-stage liver disease (n=4), protein C deficiency (n=3), paroxysmal nocturnal hemoglobinuria (PNH) (n=1), antiphospholipid syndrome (n=1) and secondary poliglobulia (n=1). In two patients the origin of BCS could not be established despite appropriate screening. Median follow-up was 29 months. Low molecular heparin with subsequent conversion to vitamin K antagonists was routinely applied in all patients. Two patients underwent TIPS procedure with good long term outcome and 10 subjects received LTx; 1 patient was lost to follow-up and 1 died of chest infection 9 years since the diagnosis of BCS was made; 14 patients, including those who received LTx, were alive and well at least one year after BCS diagnosis. All survivors remain stable and are followed-up on a regular basis.
Conclusions:
Strict adherence to the diagnostic and therapeutic guidelines plays a crucial role in the management of BCS patients. Our results confirm the efficacy of anticoagulation as well as TIPS and/or OLT in treatment of this rare condition.
Related Concept Videos
01:22Portal Hypertension
01:17Chronic Bowel Disorders: Introduction
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
01:28Esophageal Varices-II: Clinical Features and Management
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol abuse, or...