The first attempt at a human liver transplant was performed in a child with biliary atresia more than 30 years ago, but it was not until the discovery, clinical testing and adoption of cyclosporine in the late 1970's that liver transplantation began to receive acceptance worldwide. Better techniques in the donor and recipient operations, improved solutions for organ preservation and the introduction of potent immunosuppressant agents are other factors contributing to the progress of hepatic transplantation.

In children, the limiting factor is the shortage of donors. The current supply of organs is insufficient to meet the need and this problem is so serious that innovative techniques, such as the use of cutdown livers and living donor liver transplantation, have become routine procedures in large transplant centers. Considering these circumstances, the selection of patients and timing for liver transplantation is of utmost importance to ensure a successful outcome and prevent organ waste.

The goals in liver transplantation are to prolong life and to improve the quality of life. The ideal timing for transplantation depends a great deal on the underlying liver disease, the availability of other forms of therapy, the organ supply, and the expected outcome with liver transplantation. As an example, children with biliary atresia and progressive liver deterioration resulting in serious complications such as bleeding, muscle wasting, or the onset of rickets should undergo transplantation as soon as a suitable organ becomes available. On the other hand, the proper timing for a child with well compensated cirrhosis becomes more difficult to assess. In this situation, attention must be paid to subtle signs of liver failure such as a drop in the serum albumin, and increase in the prothrombin time or the development of ascites.

Diagnostic Indications

There are numerous indications for liver transplantation in children (chart); however, biliary atresia accounts for at least 50% of pediatric patients undergoing liver transplantation. The second most common reason for transplantation is a conglomerate of liver diseases which belong to inborn errors of the metabolism with alpha-1-antitrypsin deficiency being the most common among this group. Others are tyrosinemia, Wilson's disease and many others.

Contraindications

There are many contraindications or reasons why transplantation would not be performed and these are: (1) AIDS; (2) cancer outside the liver; (3) infection outside the liver; (4) technically not feasible and (5) other medical problems such as heart disease, lung failure, or epilepsy which would interfere with the success of the transplant.

Comments

Statistical formulas created by combining risk factors have been utilized as predictors of outcome. Although such formulas may be useful for the retrospective assessment of large volumes of data, they are often of little value on a daily basis. The child's condition must be assessed individually by a group of specialized physicians, including a transplant surgeon, pediatric hepatologist, and other consultants if needed. The consequences of chronic liver disease in children are devastating. Children with advanced liver disease should be referred promptly to a transplant center before they develop irreversible sequelae. Fortunately, liver transplantation is successfully performed in many medical center worldwide.

Dr. Esquivel is Professor of Surgery and Director of the Liver Transplant Program at Lucille Packard Children's Hospital at Stanford University and member of the C.L.A.S.S. Scientific Advisory Committee.