https://pubmed.ncbi.nlm.nih.gov/35051344/ PFIC

Expert Rev Gastroenterol Hepatol. 2022 Jan 20.
doi: 10.1080/17474124.2022.2032660.Online ahead of print.

Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: A Systematic Review and Meta-Analysis

Rishi Bolia 1, Akhil Dhanesh Goel 2, Vishakha Sharma 1, Anshu Srivastava 3

Abstract

Background: Biliary diversion (BD) is indicated in children with progressive familial intrahepatic cholestasis (PFIC) and pruritus refractory to medical management. Three types of BD-partial external biliary drainage (PEBD), partial internal biliary drainage (PIBD) and ileal exclusion (IE) are described. There is lack of consensus about the relative efficacy of these procedures.

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

Results: Twenty-five 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 (CI) indicated no significant differences. Absolute decrease in BA (AUROC-0.72) and bilirubin (AUROC-0.69) discriminated between responders and non-responders. Eventually, 27% required LT. PIBD fared significantly better with 10.7% (95% CI 1- 23%) undergoing LT as compared to 32% (95%CI 23-41%) with PEBD and 27% (95%CI 10-47%) with IE. The post-operative BA (AUROC-0.9) and bilirubin (AUROC-0.85) values 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% eventually need LT. Children undergoing PIBD have lower complications and need of LT as compared to other BD procedures. However, this requires caution in interpretation as the PIBD and PEBD groups differed considerably with regards to type of PFIC and duration of follow-up.