Budd Chiari syndrome - a single centre experience from the United Kingdom.

Paediatric Budd-Chiari syndrome (BCS) syndrome is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single centre retrospective review. Fourteen cases were identified, 6/14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10/14 (71%) underwent radiological intervention: 1/14 thrombolysis, 5/14 angioplasty, and 4/14 TIPS. 6/14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.