Abstract

Background: Biliary atresia (BA) is the most common indicator for liver transplant (LT) in children, however, approximately 22% will reach adulthood with their native liver, and of these, half will require transplantation later in life. The aim of this study was to analyse the surgical challenges and outcomes of patients with BA undergoing LT in adulthood. Methods: Patients with BA requiring LT at the age of 16 or older in our unit between 1989 and 2020 were included. Pretransplant, perioperative variables and outcomes were analysed. Pretransplant imaging was reviewed to assess liver appearance, spontaneous visceral portosystemic shunting (SPSS), splenomegaly, splenic artery (SA) size, and aneurysms. Results: Thirty-four patients who underwent LT for BA fulfilled the inclusion criteria, at a median age of 24 years. The main indicators for LT were synthetic failure and recurrent cholangitis. In total, 57.6% had significant enlargement of the SA, 21% had multiple SA aneurysm, and SPSS was present in 72.7% of the patients. Graft and patient survival at 1, 5, and 10 years was 97.1%, 91.2%, 91.2% and 100%, 94%, 94%, respectively Conclusions: Good outcomes after LT for BA in young patients can be achieved with careful donor selection and surgery to minimise the risk of complications. Identification of anatomical variants and shunting are helpful in guiding attitude at the time of transplant.

Highlights

  • Biliary atresia (BA) is the most common indicator for liver transplant (LT) in children

  • Categorical variables are expressed as number and percentage

  • Diagnosis of BA, early Kasai portoenterostomy (KPE), refinements of surgical technique, and centralization of care have contributed to increased survival with the native liver intact and delaying LT to later in life [1,21,22]

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Summary

Introduction

Biliary atresia (BA) is the most common indicator for liver transplant (LT) in children. 55–60% of infants undergoing KPE during the first 3 months of life clear their jaundice, two thirds will require liver transplant within 10 years or more, as they will develop liver fibrosis and cirrhosis and portal hypertension (PHT) with synthetic failure, variceal bleeding, and recurrent cholangitis. Those patients who achieve good biliary drainage after KPE reach adolescence with their native liver intact, some have clinically evident PHT and come to LT subsequently [6,7]. Identification of anatomical variants and shunting are helpful in guiding attitude at the time of transplant

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