The treatment of end-stage liver disease in childhood

With the availability of orthotopic liver transplantation as a definitive treatment option for most cases of end-stage liver disease in children, there is renewed interest in this hitherto universally fatal condition. The potential for long-term survival after liver transplantation without disability is a realistic prospect for the majority, although the surgery is one of the most difficult surgical procedures available and represents a daunting prospect. The success of liver transplantation (80% one year and approximately 70% five year survival) has required virtually a re-definition of the diagnosis of end-stage liver disease with a view to predicted outcome and timely intervention. This includes a more aggressive approach to supportive treatment in order to maintain (if not improve) the patient's clinical status, if the treatment option of transplantation is decided upon. Preferably this treatment option should be considered and offered immediately the condition of end-stage liver disease is realised, in order to allow time for full and frank discussion of treatment options, to optimize supportive therapy, to evaluate suitability for liver transplantation and to maximize prospects of obtaining a donor organ. In Australia, particularly in children, liver transplantation is limited by the scarcity of suitable donor organs, although the development of reduction hepatectomy of an adult organ has allowed access to a much larger donor pool. This development has particular advantages in Australia, allowing organs from 80 kg adults to be transplanted successfully into 5 kg or 6 kg infants and has, at the time of writing, virtually eliminated potential paediatric recipient deaths while awaiting a suitable donor.