Partial External Biliary Diversion in Children With Progressive Familial Intrahepatic Cholestasis and Alagille Disease

Background: Partial external biliary diversion (PEBD) is a promising treatment for children with progressive familial intrahepatic cholestasis (PFIC) and Alagille disease. Little is known about long-term Outcomes. Patients and Methods: A retrospective chart review of all patients undergoing PEBD in the University Medical Centre of Groningen (UMCG). Results: Between 2000 and 2005. PEBD was performed on 14 children with severe pruritus (PFIC I I. mean age 5.3 +/- 4.4 years: Alagille 3, mean age 7.4 +/- 4.2 years). Stature was <-2 standard deviation score (SDS) in 50%. Median preoperative Serum bile salt concentration was 318 mu mol/L (range 23-527 mu mol/L). Twenty-nine percent had severe liver fibrosis and 71% had mild or moderate fibrosis. Median follow-up was 3.1 years (range 2.0-5.7 years). One patient (7%) underwent a liver transplantation at 3.2 years post-PEBD. Two years postoperatively, 50% were without pruritus and 21% had mild pruritus. In 29%, pruritus had not diminished; 3 of them had severe fibrosis preoperatively. In patients with mild or moderate fibrosis, PEBD decreased serum bile salts (105 mu mol/L [range 8-269 mu mol/L] 2 years postoperatively). Bile salts did not decrease in the patients with severe fibrosis. Two years after PEBD, 27% had a stature below -2 SDS. Conclusions: At median follow-up of 3.1 years after PEBD, pruritus has been relieved in 75%. Bile salts level and growth are improved in most patients. Longer follow-up is needed to determine whether PEBD can postpone or avoid the demand for fiver transplantation. JPGN 49:216-221, 2009.