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Primary Budd-Chiari Syndrome in Children: King's College Hospital Experience
Susana Nobre1, Rajeev Khanna, Natalie Bab
1*Paediatric Liver, GI and Nutrition Center†Paediatric Hematology Unit‡Department of Radiology§Liver Transplant Service Institute of Liver Studies, King's College Hospital, London, UK.
Insights
Primary Budd-Chiari syndrome in children is rare but manageable. Early anticoagulation and interventions like shunts or liver transplant offer good long-term outcomes despite high initial risks.
Area of Science:
- Pediatric Hepatology
- Vascular Liver Disease
- Thrombotic Disorders
Background:
- Primary Budd-Chiari syndrome (PBCS) is an uncommon cause of pediatric liver disease in Western countries.
- PBCS involves the obstruction of hepatic venous outflow, leading to liver damage.
Purpose of the Study:
- To review the clinical presentation, management, and outcomes of pediatric patients with Primary Budd-Chiari syndrome.
- To evaluate the effectiveness of various treatment modalities in this cohort.
Main Methods:
- Retrospective review of seven pediatric patients diagnosed with PBCS between January 2001 and November 2015.
- Analysis of patient demographics, clinical presentation, predisposing factors for thrombosis, treatments received, and follow-up data.
Main Results:
- Most children presented with chronic symptoms and had identifiable predisposing factors for thrombosis.
- Treatments included anticoagulation, radiological interventions (transjugular intrahepatic portosystemic shunts, hepatic vein stenting), liver transplantation, mesocaval shunt, and bone marrow transplantation.
- One patient died 3 years post-liver transplant due to subarachnoid hemorrhage; others showed good recovery at a median 6-year follow-up.
Conclusions:
- Primary Budd-Chiari syndrome in children, though serious, can achieve favorable long-term outcomes.
- A multidisciplinary approach integrating medical management, interventional radiology, and surgical options is crucial for successful treatment.
Abstract:
Primary Budd-Chiari syndrome is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary Budd-Chiari syndrome presenting from January 2001 to November 2015 to our hospital. Seven children were identified. Their presentation was mostly chronic. All had predisposing factors for thrombosis and were started on anticoagulation. Radiological interventions (2 transjugular intrahepatic portosystemic shunts and 1 hepatic vein stenting), liver transplant and mesocaval shunt were done in 3, 2, and 1 patients, respectively; 1 child underwent bone marrow transplantation following transjugular intrahepatic portosystemic shunts and 1 child was managed only medically. After liver transplantation, one child died 3 years later as a result of subarachnoid haemorrhage, whereas others remain well at a median follow-up of 6 years. Despite high morbidity, the disease can have a good long-term outcome with a multidisciplinary approach.