Technical success and outcomes in pediatric patients undergoing transjugular intrahepatic portosystemic shunt placement: a 20-year experience.

ABSTRACT

BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) placement has been extensively studied in adults. The experience with TIPS placement in pediatric patients, however, is limited. OBJECTIVE: The purpose of this study was to report technical success and clinical outcomes in pediatric patients undergoing TIPS placement. MATERIALS AND METHODS: Twenty-one children - 12 (57%) boys and 9 (43%) girls, mean age 12.1 years (range, 2-17 years) - underwent TIPS placement from January 1997 to January 2017. Etiologies of hepatic dysfunction included biliary atresia (n=5; 24%), cryptogenic cirrhosis (n=4; 19%), portal or hepatic vein thrombosis (n=4, 14%), autosomal-recessive polycystic kidney disease (n=3; 14%), primary sclerosing cholangitis (n=2; 10%) and others (n=3, 14%). Indications for TIPS placement included variceal hemorrhage (n=20; 95%) and refractory ascites (n=1; 5%). Technical success, manometry findings, stent type, hemodynamic success, complications, liver enzymes, and clinical outcomes were recorded. RESULTS: TIPS placement was technically successful in 20 of 21 (95%) children, with no immediate complications. Mean pre- and post-TIPS portosystemic gradient was 18.5±10.7 mmHg and 7.1±3.9 mmHg, respectively. Twenty-two total stents were successfully placed in 20 children. Stents used included Viatorr (n=9; 41%), Wallstent (n=7; 32%), Express (n=5; 23%), and iCAST (n=1; 5%). All children had resolution of variceal bleeding or ascites. TIPS revision was required in 9 (45%) children, with a mean of 2.2 revisions. Hepatic encephalopathy developed in 10 children (48%), at a mean of 223.7 days following TIPS placement. During the study, 6 (29%) children underwent liver transplantation. CONCLUSION: TIPS placement in pediatric patients has high technical success with excellent resolution of variceal hemorrhage and ascites. TIPS revision was required in nearly half of the cohort, with hepatic encephalopathy common after shunt placement.