Techniques and Outcomes of Transjugular Intrahepatic Portosystemic Shunting in Infants with Budd-Chiari Syndrome

Abstract

PurposeTo describe the technical aspects, feasibility, and outcomes in children with Budd-Chiari syndrome (BCS) undergoing transjugular intrahepatic portosystemic shunt (TIPS) creation during infancy. Materials and MethodsA retrospective review of infants with BCS undergoing TIPS creation between January 2012 and December 2018 was performed. Eight infants (5 males) underwent TIPS creation (7 for refractory ascites and 1 for refractory variceal bleeding) during the study period. The median age at TIPS creation was 10.5 months (range, 8–16 months). The median elapsed time between presentation and TIPS creation was 6.5 months (range, 0–13 months). The median weight and median pediatric end-stage liver disease score of the infants at the time of TIPS creation were 6.7 kg (range, 5.4–10 kg) and 13 kg (range, 8–18 kg), respectively. ResultsTIPS creation was successful in all patients. There were no immediate postprocedural complications. An 18-gauge hollow needle was manually curved, through which a 21-gauge Chiba needle was inserted to access the portal vein. All patients received 1 or 2 overlapping bare metal stents. One patient was lost to follow-up after the procedure. The median follow-up duration was 32 months (range, 14–51 months). Four of 7 infants needed reintervention. Two children died during the follow-up period. Two children successfully underwent living donor liver transplant, whereas the remaining 3 children were asymptomatic at the follow-up. ConclusionsTIPS creation was found to be safe and efficacious in improving portal hypertension and growth in these children, although, with a higher rate of reinterventions, possibly due to the use of small, bare metal stents.