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

Abstract

Prior to 1955, when Morio Kasai first performed the hepatic portoenterostomy procedure which now bears his name, Biliary atresia (BA) was a uniformly fatal disease. Both the Kasai procedure and liver transplantation have markedly improved the outlook for infants with this condition. Although long-term survival with native liver occurs in the minority, survival rates post liver transplantation are high. Most young people born with BA will now survive into adulthood but their ongoing requirements for health care will necessitate their transition from a family-centred paediatric service to a patient-centred adult service. Despite a rapid growth in transition services over recent years and progress in transitional care, transition from paediatric to adult services is still a risk for poor clinical and psychosocial outcomes and increased health care costs. Adult hepatologists should be aware of the clinical management and complications of biliary atresia and the long-term consequences of liver transplantation in childhood. Survivors of childhood illness require a different approach to that for young adults presenting after 18 years of age with careful consideration of their emotional, social, and sexual health. They need to understand the risks of non-adherence, both for clinic appointments and medication, as well as the implications for graft loss. Developing adequate transitional care for these young people is based on effective collaboration at the paediatric–adult interface and is a major challenge for paediatric and adult providers alike in the 21st century. This entails education for patients and adult physicians in order to familiarise them with the long-term complications, in particular for those surviving with their native liver and the timing of consideration of liver transplantation if required. This article focusses on the outcome for children with biliary atresia who survive into adolescence and adult life with considerations on their current management and prognosis.

Long‐term clinical and socioeconomic outcomes of children with biliary atresia

“…As such, adult hepatologists should be aware of the clinical management and complications of BA and the long-term consequences of LT in childhood. 22 This study encompasses the management of BA patients in a single center over four decades. This is a unique cohort of large numbers of BA patients, given that the numbers of babies born with BA in the United Kingdom are less than 50 per year.…”

“…Since most young people born with BA will now survive into adulthood, ongoing requirements for health care will necessitate their transition from a family‐centered pediatric service to a patient‐centered adult service. As such, adult hepatologists should be aware of the clinical management and complications of BA and the long‐term consequences of LT in childhood 22 …”

“…As such, adult hepatologists should be aware of the clinical management and complications of BA and the long‐term consequences of LT in childhood. 22 …”