Budd-Chiari Syndrome—A Single Center Experience From the United Kingdom

Abstract

Pediatric Budd-Chiari syndrome (BCS) 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 center retrospective review. Fourteen cases were identified; 6 of 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 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 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.

Post-Transjugular Intrahepatic Portosystemic Shunt (TIPS) Hepatic Encephalopathy—A Review of the Past Decade’s Literature Focusing on Incidence, Risk Factors, and Prophylaxis

“…The indications for TIPS placement have been expanded and refined in recent years. Preemptive TIPS is now the new standard in many centres, and indications are broadened to include non-cirrhotic portal hypertension, portal vein thrombosis, Budd–Chiari syndrome, hepatorenal and hepatopulmonal syndrome, and cases of hepatocellular carcinoma with portosystemic symptoms [ 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ]. Although technical advancements have decreased post-TIPS morbidity significantly, hepatic encephalopathy (HE) remains a well-known and debilitating complication of TIPS placement (HE) even when present as minimal or grade I HE (‘covert HE’) [ 10 , 11 , 12 , 13 ].…”