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[Chronic Budd-Chiari syndrome can cause liver cirrhosis]
Stine Karlsen1, Dennis Tønner Nielsen, Henning Grønbæk
1Medicinsk Afdeling V, Hepatologi og Gastroenterologi, Aarhus Universitetshospital, Nørrebrogade 44, 8000 Aarhus C, Denmark. stinkarl@rm.dk
Insights
Budd-Chiari syndrome (BCS), a rare liver disease, can cause severe ascites mimicking cancer. Successful transjugular intrahepatic portosystemic shunt (TIPS) relieved symptoms in a challenging case.
Area of Science:
- Hepatology
- Vascular Medicine
- Interventional Radiology
Background:
- Budd-Chiari syndrome (BCS) is a rare hepatic vascular disorder characterized by obstruction of the hepatic venous outflow tract, leading to congestive hepatopathy.
- Clinical manifestations include ascites, hepatomegaly, abdominal pain, and liver dysfunction, often resulting from thrombosis, malformations, or venous compression.
Observation:
- A challenging case of liver cirrhosis with refractory ascites was initially suspected to be hepatocellular carcinoma due to nodular appearance.
- Diagnostic confirmation of BCS was achieved during the transjugular intrahepatic portosystemic shunt (TIPS) procedure.
Findings:
- The patient's BCS was complicated by liver cirrhosis and refractory ascites, posing a diagnostic challenge.
- Successful vascular stenting during the TIPS procedure effectively resolved the ascites formation.
Implications:
- This case highlights the importance of considering BCS in the differential diagnosis of refractory ascites, even when mimicking malignancy.
- Transjugular intrahepatic portosystemic shunt (TIPS) with vascular stenting offers a successful therapeutic option for managing ascites in BCS.
- Accurate diagnosis and timely intervention are crucial for improving outcomes in patients with Budd-Chiari syndrome.
Abstract:
Budd-Chiari syndrome (BCS) is a rare disease defined by congestive hepatopathy with obstruction of the hepatic venous outflow tract. Classical symptoms and signs include ascites, hepatomegaly, abdominal pain and various degrees of liver dysfunction. BCS is predominantly caused by thrombosis, malformations and venous compression. We present a case, in which BCS was the cause of liver cirrhosis complicated with refractory ascites and which can be misinterpreted as hepatocellular carcinoma nodules. The diagnosis was confirmed during the transjugular intrahepatic portosystemic shunt procedure with successful vascular stenting resolving the ascites formation.
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