Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy

Patrick Ho Yu Chung,Albert Chi Yan Chan,Fanny Yeung,Kim Hung Lee,Edwin Kin Wai Chan,Judy Wing Suet Hung,Kenneth Kak Yuen Wong,Paul Kwong Hang Tam,Michael Wai Yip Leung,Jennifer Wai Cheung Mou

doi:10.1038/s41598-021-90860-w

Abstract

We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications. Factors affecting the operative outcomes were analyzed. The median duration of follow up was 17.5 (IQR: 13.5–22) years. Over 66% of patients became jaundice-freed at 1 year after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 (IQR: 4.3–8.4) years. The NLS rates at 10 and 20 years were 70.7% and 61.5% respectively with no significant change over the study period. The median age at KPE was 59 (IQR: 49–67) days. KPE performed before 70 days was associated with higher odd ratios for successful drainage but the age of KPE did not have an impact on the long-term NLS. Among all native liver survivors (n = 153), the median bilirubin level was 24 (IQR: 16–36) µmol/L. Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively. With a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long-term survival without LT. Although cholestasis, portal hypertension and recurrent cholangitis are common in long-term NLS, with a comprehensive follow management strategy, they do not always necessitate LT. Our study serves as an example for countries where deceased donor organs are scarce due to very low donation rate.

Highlights

We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE)
KPE is labelled as failure when patients develop complications related to recurrent cholangitis, portal hypertension (PHT) and hepatic dysfunction which could happen in 60% of BA patients[2]
We observed a higher incidence between 2006 and 2015 and this could be due to the transient immigration policy that allowed eligible women from Mainland China, one of the countries with the highest incidence of BA, to deliver their babies in Hong Kong during that period

Summary

Introduction

We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). Abbreviations BA Biliary atresia COJ Clearance of jaundice KPE Kasai portoenterostomy LT Liver transplant MELD Model for end-stage liver disease NLS Native liver survival OGV Oesophago-gastric varices PELD Pediatric end-stage liver disease PHT Portal hypertension. LT is a potential treatment for these complications, transplant recipients need to face the problems associated with an ultra-major operation and the life-long use of immunosuppressants This could impair the immune system, leading to recurrent infection and most severely, haematological m alignancy[7]. The main purposes of this study were (1) to describe our territory-wide experience in managing BA and report the treatment outcomes of KPE based on 37 years’ follow up data; and (2) to present the long-term problems encountered by native liver survivors and discuss management strategies other than LT

Methods

Results

Conclusion