Budd-Chiari syndrome
Pieter Martens1, Frederik Nevens2
1Department of Internal Medicine.
Insights
Budd-Chiari syndrome (BCS) involves hepatic outflow obstruction, often linked to prothrombotic risk factors like myeloproliferative disorders. Early diagnosis and anticoagulation, followed by interventions if needed, lead to a 90% five-year survival rate.
Area of Science:
- Hepatology
- Vascular Medicine
- Hematology
Background:
- Budd-Chiari syndrome (BCS) is a rare hepatic disorder caused by obstruction of hepatic venous outflow.
- It is frequently associated with underlying prothrombotic risk factors, most commonly myeloproliferative disorders.
- BCS presents diversely, mimicking other acute and chronic liver diseases, necessitating a high index of suspicion in patients with risk factors.
Purpose of the Study:
- To outline the diagnostic approach and management strategies for Budd-Chiari syndrome.
- To emphasize the importance of identifying and managing underlying prothrombotic risk factors.
- To discuss the efficacy of various treatment modalities and long-term outcomes.
Main Methods:
- Diagnosis confirmed by Doppler ultrasound, with CT or MRI for further assessment.
- Primary treatment involves anticoagulation.
- Interventional strategies include angioplasty, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation for refractory cases.
Main Results:
- Anticoagulation is the cornerstone of BCS management.
- The majority of patients require additional invasive treatments beyond anticoagulation.
- Proposed treatment algorithms guide interventions based on treatment response, achieving a five-year survival rate of 90%.
Conclusions:
- Budd-Chiari syndrome requires prompt diagnosis and management of underlying prothrombotic conditions.
- A multidisciplinary approach integrating anticoagulation and interventional procedures improves patient survival.
- Long-term surveillance is crucial to monitor for recurrence and potential complications such as myeloproliferative disorder progression or post-transplant lymphoproliferative disorders.
Abstract:
Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract at any level between the junction of the inferior vena cava with the right atrium and the small hepatic veins. In the West, BCS is a rare hepatic manifestation of one or more underlying prothrombotic risk factors. The most common underlying prothrombotic risk factor is a myeloproliferative disorder, although it is now recognized that almost half of patients have multiple underlying prothrombotic risk factors. Clinical manifestations can be diverse, making BCS a possible differential diagnosis of many acute and chronic liver diseases. The index of suspicion should be very low if there is a known underlying prothrombotic risk factor and new onset of liver disease. Doppler ultrasound is sufficient for confirming the diagnosis, although tomographic imaging (computed tomography (CT) or magnetic resonance imaging (MRI)) is often necessary for further treatment and discussion with a multidisciplinary team. Anticoagulation is the cornerstone of the treatment. Despite the use of anticoagulation, the majority of patients need additional (more invasive) treatment strategies. Algorithms consisting of local angioplasty, TIPS and liver transplantation have been proposed, with treatment choice dictated by a lack of response to a less-invasive treatment regimen. The application of these treatment strategies allows for a five-year survival rate of 90%. In the long term the disease course of BCS can sometimes be complicated by recurrence, progression of the underlying myeloproliferative disorder, or development of post-transplant lymphoma in transplant patients.
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