COMBINED HEART-LUNG-LIVER, DOUBLE LUNG-LIVER, AND ISOLATED LIVER-TRANSPLANTATION FOR CYSTIC-FIBROSIS IN CHILDREN

Between June 1990 and September 1995, 8 of 24 children with cystic fib rosis (CF) who were accepted either for combined transplantation or is olated liver transplantation died while waiting for a graft; 11 underw ent 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) un derwent isolated liver transplantation (three full-size livers, one pa rtial liver). There was one perioperative death in each group. Pulmona ry infection was the most common cause of morbidity in group 1. Other complications in group 1 included tracheobronchial stenosis (n = 2), b iliary stricture (n = 2), and severe ascites (n = 2). All were success fully treated. Obliterative bronchiolitis developed in three patients. This was treated with FK 506. In group 2, pulmonary function tests im proved or remained stable after liver transplantation. Surgical compli cations 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.