Liver Transplantation for Biliary Atresia in Adulthood: Single-Centre Surgical Experience

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.

Biliary atresia and liver transplantation in the United States: A contemporary analysis

“…In the older group, BA LT recipients had superior graft outcomes compared to older non‐BA LT recipients. These excellent graft outcomes among older BA LT recipients is consistent with the existing literature 35 . The difference in graft survival rates between the two groups may be explained in part by the large proportion of PSC LT recipients in the older non‐BA cholestatic group.…”

UK guideline on the transition and management of childhood liver diseases in adulthood

“…For those requiring liver transplantation in adulthood, medical and surgical teams should be aware of the anatomical variants associated with BA and the poor sensitivity of prognostic outcome models such as the model for end-stage liver disease (MELD) and the United Kingdom Model for End-Stage Liver Disease (UKELD). 94,95 Neurocognitive outcomes are known to be inferior to that of healthy peers even in those undergoing transplantation at a young age and are likely to affect educational and social outcomes. 96…”

Biliary Atresia in Adolescence and Adult Life: Medical, Surgical and Psychological Aspects

“…In contrast to adults with cirrhosis, hepatocellular carcinoma was rare (1.3%). Synthetic liver failure, recurrent cholangitis and complications of portal hypertension were the main indications for Liver transplantation [ 50 , 51 ].…”

“…Less information on its prevalence in adolescence or adulthood is available. In our series of 89 young people with BA surviving with their native liver after the age of 16 years, 10% developed at least one episode of cholangitis between the age of 12 and 16 years and this was found to be associated with a 4-fold risk of requiring liver transplantation during adulthood [ 51 ] The symptoms can be non-specific and liver function tests not helpful as 90% of BA patients will have some deranged liver function tests. Cholangitis should be suspected when presenting with right upper quadrant pain, general malaise and nausea.…”

“…Adults with BA requiring LT also provide surgical challenges in particular related to the vascular system. In a review of 36 adults with BA undergoing LT, 57.6% was found to have significant enlargement of the splenic artery with 21% noted to have multiple splenic artery aneurysms [ 51 ]. Spontaneous visceral porto-systemic shunting (SPSS) was present in 72.7%.…”