Transjugular intrahepatic portosystemic shunt in five children with cystic fibrosis: long-term results.

BACKGROUND/AIMS: The aim of this study was to report results of TIPS (transjugular intrahepatic portosystemic shunt) in 5 consecutive children with cystic fibrosis and monitor long-term follow-up results.

METHODOLOGY

Five cystic fibrosis patients with multinodular cirrhosis of the liver and complications of portal hypertension (repeated variceal bleeding refractory to endoscopic treatment) underwent TIPS creation. There were 3 males and 2 females aged 8 to 18 years (median 14 yrs), their weight range was 27-51 (median 40) kg. A routine TIPS technique was used. The patients were followed by ultrasonography.

RESULTS

The TIPS was successfully performed in all five patients. The mean portosystemic pressure gradient was reduced from 17 to 10 mmHg. There were no deaths related to the procedure. No clinical or laboratory signs of bleeding into the gastrointestinal tract were observed in any patient within the first 30 days following TIPS. There was recurrent bleeding 6 times and asymptomatic stenoses were revealed by ultrasonography 15 times during a follow-up period of 15-81 (median 70) months. All stenoses were successfully dilated. One patient had liver transplantation 15 months after TIPS. Two patients died 6.7 years and 4.5 years following the placement of TIPS due to respiratory insufficiency.

CONCLUSIONS

Symptomatic portal hypertension was successfully managed with TIPS in long-term follow-up; multiple reinterventions were required for shunt stenoses. TIPS served as a bridge to liver transplantation in one case.