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

PURPOSE

To 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 METHODS

A 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.

RESULTS

TIPS 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.

CONCLUSIONS

TIPS 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.