Biliary diversion in progressive familial intrahepatic cholestasis: a systematic review and meta-analysis

Abstract

Background: Biliary diversion (BD) is indicated in progressive familial intrahepatic cholestasis (PFIC) with refractory pruritus. Three types-partial external biliary drainage (PEBD), partial internal biliary drainage (PIBD), and ileal exclusion (IE) are described, with no consensus about the relative efficacy of these procedures.

Methods: PubMed, Scopus, and Google Scholar were searched for publications on PFIC and BD. Improvement in pruritus, serum bile acid (BA), and need for liver transplantation (LT) were compared between the various BD procedures.

Results: 25 studies [424 children (PEBD-301, PIBD-93, IE-30)] were included. Pruritus resolved in 59.5% [PIBD:72% (95%CI 43-96%), PEBD:57% (95%CI 43-71%) and IE:48% (95%CI 14-82%)] cases. Significant overlap in confidence intervals indicated no significant differences. Absolute decrease in BA (AUROC-0.72) and bilirubin (AUROC-0.69) discriminated responders and non-responders. Eventually, 27% required LT: PIBD 10.7%, PEBD32%, IE 27%. The post-operative BA (AUROC-0.9) and bilirubin (AUROC-0.85) determined need for LT. Complications were commoner in PEBD than PIBD (38% vs 21.8%: p=0.02).

Conclusion: 59.5% children have pruritus relief after BD and 27% need LT. PIBD has lower complications and LT requirement than PEBD. However, this requires cautious interpretation as the 2 groups differed in PFIC type and follow-up duration.

Keywords: Pruritus; biliary diversion; cholestasis; liver transplantation; progressive familial intrahepatic cholestasis.