Liver transplantation for biliary atresia

Abstract

Orthotopic liver transplantation was performed 15 months to 20 years ago in 126 recipients, all of whom were under 18 years of age. Eighty-six of these pediatric recipients were treated before 1980 with azathioprine (or cyclophosphamide) and prednisone, to which antilymphocyte globulin (ALG) usually was added. One-year patient survival was 40%. In the last 40 cases, the new drug cyclosporine has been given with low doses of steroids. The one-year patient survival increased to 65%. Both in the pre-cyclosporine era and more recently, the survival of patients with biliary atresia has been lower than in the next largest category of patients, namely, those with liver-based inborn metabolic errors. The difficulty of operation in patients with biliary atresia has been greater than in recipients with other diagnoses, partly because of previous operations such as portoenterostomy (Kasai procedure). Hepatic portoenterostomy, worthwhile as it is, has posed technical difficulties for eventual liver transplantation, particularly when complicated Roux limb techniques or venting procedures have been applied. In our total experience the longest survival after liver replacement in a child whose original diagnosis was biliary atresia is 13⅔ years.

Fig. 1

A comparison of survival after liver transplantation between 86 pediatric liver recipients under conventional immunosuppressive therapy (see text for description) and 40 pediatric recipients under cyclosporineprednisone therapy.

Fig. 2

Influence of original liver disease on 1-year survival under conventional immunosuppression.

Fig. 3

Influence of original liver disease on 1-year survival under cyclosporine-steroid therapy.

Fig. 4

Age distribution of 126 pediatric liver recipients. A shaded square represents a child who survived more than 3 months, and a black square represents a child who died within 3 months. All of the surviving patients treated with cyclosporine have follow-ups of at least 15 months.