Primary Liver Transplant in Biliary Atresia: The Case for and Against.

The role of liver transplantation as a primary procedure in biliary atresia has been argued over for at least 40 years, indeed since the coming of age of safe liver transplantation during the 1980s. Yet, it is not a common option in most series (usually ≤5%) and typically reserved for those with late presentations (arguably >100 days) with established cirrhosis. This review presents the pros and cons of primary liver transplant. The pros are based upon the observation that at best a Kasai portoenterostomy (KPE) is simply palliative in most, and at worse has no effect whatsoever on restoration of bile flow and is therefore pointless. Set against this are the cons: there is a dearth of prognostic tests (clinical, biochemical, or histological) at the time of presentation which may predict inevitable failure; the possibility of long-term native liver survival to adulthood in a proportion (albeit a minority); and the implied increased need for donor organs suitable for infants - a stressor for an already overstressed system. Improving results from KPE in terms of increasing the proportions clearing their jaundice and minimizing the effects of chronic liver fibrosis and cirrhosis would surely limit the siren calls for primary transplants but the key must be better discrimination at presentation with the use of biomarkers (circulatory or histological, individually or together) to enable better decision making.