Young People With Biliary Atresia Requiring Liver Transplantation: A Distinct Population Requiring Specialist Care

Young people (YP) born with biliary atresia (BA) are an emerging population for adult hepatologists with 40% to 45% of children entering adolescence with their native liver intact. For those requiring liver transplantation (LT) during adolescence, disparity on the waiting list and post-LT outcome for young adults compared with younger and older age groups has stimulated discussion about the optimal timing of listing. In this study, we review our experience of YP with BA requiring LT during adolescence and young adulthood.

Retrospective, single-center review of patients with BA requiring LT > 11 years.

Thirty-six YP (16 male) underwent LT between 1991 and 2014 at a median age of 16.6 (interquartile range [IQR], 14.2 to 19.5) years. The commonest indications for listing were refractory cholangitis (31%), synthetic failure (25%), and variceal bleeding (14%). Patients listed by the adult team (n = 14) waited longer than those listed by the pediatric team (10 [IQR, 7.7 to 24.6] vs 5.8 [IQR, 4.0 to 15.1] months; P < 0.05) and were more likely to require intensive care support at time of listing (29% vs 5%; P < 0.05). Admission to intensive care unit at listing was associated with poorer patient and graft survival and support from a multidisciplinary liver transition service improved survival. Liver disease severity scores did not correlate with time on waiting list or outcome.

YP with BA requires close monitoring by specialists familiar with their condition and timing for LT needs to be fine-tuned to avoid clinical decompensation and improve long-term outcomes.