Primary Budd-chiari Syndrome in Children: King's College Hospital Experience

Primary Budd-Chiari syndrome (BCS) is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary BCS presenting from Jan 2001 to Nov 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 (TIPSS) and 1 hepatic vein stenting), liver transplant and mesocaval shunt were done in 3, 2 and 1 patients, respectively; one child underwent bone-marrow transplantation following TIPSS, and one child was managed only medically. Post liver transplantation, one child died 3 years later due to subarachnoid haemorrhage while others remain well at a median follow-up of 6 years. In spite of the high morbidity, the disease can have a good long term outcome with a multidisciplinary approach.