Combined heart-lung-liver, double lung-liver, and isolated liver transplantation for cystic fibrosis in children

Abstract Between June 1990 and September 1995, 8 of 24 children with cystic fibrosis (CF) who were accepted either for combined transplantation or isolated liver transplantation died while waiting for a graft; 11 underwent transplantation and 5 are currently on the waiting list. Of the 11 children who had surgery, 7 (group 1) underwent one of the following procedures: heart-lung-liver ( n = 4), sequential double lung-liver ( n = 2), or bilateral lobar lung from a split left lung and reduced liver ( n = 1). During the same period, the four other children (group 2) underwent isolated liver transplantation (three full-size livers, one partial liver). There was one perioperative death in each group. Pulmonary infection was the most common cause of morbidity in group 1. Other complications in group 1 included tracheobronchial stenosis ( n = 2), biliary stricture ( n = 2), and severe ascites ( n = 2). All were successfully treated. Oblit-erative bronchiolitis developed in three patients. This was treated with FK 506. In group 2, pulmonary function tests improved or remained stable after liver transplantation. Surgical complications in group 2 included severe ascites ( n = 1), biliary stricture ( n = 1), and abscess of the liver ( n = 1). Actuarial survival was 85.7 %% 2 % in group 1 at 1 year; it remained unchanged at 3 years and was 64.2 % at 5 years.