Liver Transplantation in Children: Update 2010

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Historical notes

Thomas E. Starzl performed the first liver transplantation in 1963 in a 3-year-old child with biliary atresia and thus pioneered a heroic journey through surgical refinement and improved immune suppression.3 Although this first child died because of surgical difficulties and coagulopathy, Dr Starzl persisted and in the late 1960s performed another 8 pediatric liver transplants of which all the children survived surgery. Unfortunately, initial survival rates were poor because of inadequate

Indications for listing

There are 4 broad listing indications for evaluation and listing for pediatric liver transplantation (Table 1). The primary indication is the onset of life-threatening complications secondary to hepatic failure or chronic end-stage liver disease. Progressive primary liver disease refractory to maximal medical management is also an indication for liver transplantation, before the development of life-threatening complications. A smaller number of liver transplants are performed for metabolic

Contraindications to transplantation

It is important to identify contraindications to liver transplantation at the earliest stage of the evaluation process. In pediatric transplantation, there are very few absolute contraindications. These would include conditions in which liver transplantation is futile and will not improve overall survival or quality of life, and this list of conditions has shortened dramatically over the years (Box 1). These conditions are largely extrahepatic diseases in which liver transplantation cannot

Evaluation of recipient

The appropriate selection and evaluation of potential recipients is fundamental in achieving the level of liver transplant success described earlier. The initial purpose of detailed evaluation of a candidate is to determine that liver transplantation remains the best option and no other medical therapies could be life sustaining with adequate quality of life. Other goals of a complete evaluation are to maximize nutrition, finesse medical therapy, provide education and support to the patient and

Donor options

All potential options for transplantation should be discussed with the family at the time of evaluation, including deceased donor transplantation with appropriate size donor, split liver transplantation from a young adult donor, and living donor transplantation. The possibility of a living donor should be introduced during the evaluation process, if determined by the transplant team to be an appropriate option. It is important to provide an objective presentation of living donation with a clear

Organ allocation

Organ allocation is a complex process guided by principles of equity, justice, utility, and benefit, in an era when there is a persistent donor shortage. In most countries with an established liver transplantation network, similar concepts arise with an understanding that organs need to be allocated to the sickest patients. Thus, there are special considerations for patients with fulminant liver failure and malignancy. In addition, most systems preferentially allocate pediatric donors to

Transplant surgery

It is not possible to review the details of liver transplant surgery here, however it is important for any health care professional caring for these children to have a basic understanding of the processes involved. Liver transplantation has 3 major phases beginning with the recipient hepatectomy. In pediatric patients at least half have previously undergone abdominal surgery (portoenterostomy or Kasai) and this is often the most difficult part of the transplant because of adhesions, and

Graft dysfunction

There are multiple causes of graft dysfunction after liver transplantation and the relative likelihood of each of these varies according to the time since surgery and the clinical context. An exhaustive discussion of graft dysfunction is not possible here and the following is intended as a simple overview.

Risk factors and outcomes

The overall results from pediatric liver transplantation are exemplary. OPTN data reveal 1- and 5-year patient survival rates of 83% to 91% and 82% to 84%, respectively. These survival rates vary according to the age at transplantation. Although survival for children less than 1 year old has improved dramatically, they still represent the lower end of the range. The underlying diagnosis at transplantation also has an effect on outcomes. Patients with acute liver failure have worse early and

Summary

Pediatric liver transplantation is a well-established and successful strategy. Much of the discussion in this article has focused on the management of peri- and posttransplant patients. Another area of concern for the transplant community is the supply of available donors. In 2006 there were more than 350 children waiting for a liver transplant and children less than 6 years old have the highest death rate of all candidates.49 Surgical advances to split organs and living related donation has

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References (51)

  • K.A. Soltys et al.

    Late graft loss or death in pediatric liver transplantation: an analysis of the SPLIT database

    Am J Transplant

    (2007)
  • K.M. Campbell et al.

    High prevalence of renal dysfunction in long-term survivors after pediatric liver transplantation

    J Pediatr

    (2006)
  • E. Varo et al.

    Cardiovascular risk factors in liver allograft recipients: relationship with immunosuppressive therapy

    Transplant Proc

    (2002)
  • E.M. Alonso et al.

    Linear growth patterns in prepubertal children following liver transplantation

    Am J Transplant

    (2009)
  • L.E. Bell et al.

    Adolescent transition to adult care in solid organ transplantation: a consensus conference report

    Am J Transplant

    (2008)
  • J.C. Magee et al.

    Pediatric transplantation in the United States, 1997–2006

    Am J Transplant

    (2008)
  • P.L. Abt et al.

    Donation after cardiac death in the US: history and use

    J Am Coll Surg

    (2006)
  • D.A. Kelly

    Current issues in pediatric transplantation

    Pediatr Transplant

    (2006)
  • J.B. Otte

    History of pediatric liver transplantation. Where are we coming from? Where do we stand?

    Pediatr Transplant

    (2002)
  • National Institutes of Health Consensus Development Conference Statement: liver transplantation–June 20–23, 1983

    Hepatology

    (1984)
  • V.L. Ng et al.

    Outcomes of 5-year survivors of pediatric liver transplantation: report on 461 children from a North American multicenter registry

    Pediatrics

    (2008)
  • M.R. Narkewicz et al.

    Pattern of diagnostic evaluation for the causes of pediatric acute liver failure: an opportunity for quality improvement

    J Pediatr

    (2009)
  • Y. Futagawa et al.

    An analysis of the OPTN/UNOS Liver Transplant Registry

    Clin Transplant

    (2004)
  • P. Baliga et al.

    Posttransplant survival in pediatric fulminant hepatic failure: the SPLIT experience

    Liver Transpl

    (2004)
  • S. Florman et al.

    Living-related liver transplantation in inherited metabolic liver disease: feasibility and cautions

    J Pediatr Gastroenterol Nutr

    (2001)
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