Earlier identification of biliary atresia and hepatobiliary disease: selective screening in the third week of life.

The possibility of screening for biliary atresia has emerged as a controversial issuel-6 in the wake of Yellow Alert, an educational programme launched in May 1993, by the Children's Liver Disease Foundation with the help of the Department of Health, in order to improve the outcome of hepatobiliary disease in infants. The aim of that campaign is to make certain that all babies who remain jaundiced after two weeks of age are tested for conjugated hyperbilirubinaemia and referred for timely specialist investigation and treatment if positive. Such infants most commonly have disorders other than biliary atresia for which early and specific treatment is equally desirable. All have vitamin K malabsorption and are at special risk of life threatening bleeding or brain damage from vitamin K responsive haemorrhagic disease, a risk that will almost certainly be increased by the decline in the use of parenteral vitamin K in the newborn.7 We welcome the interest as an opportunity to achieve a consensus that can be implemented nationally on how best to ensure early identification and referral for diagnosis and treatment of infants with biliary atresia and other hepatobiliary disorders starting in the first weeks of life. The main issues raised with respect to screening for biliary atresia are whether the condition is sufficiently important to merit treatment and whether earlier treatment is so much better than late that it satisfies criteria for inclusion in a screening programme. If it is, should screening be delayed to 21 days of age to be more cost effective and create less parental anxiety? As screening involves seeking out and testing the whole population, we maintain that assessing persistent jaundice in the neonatal period might be considered selective screening or more correctly case finding, that is, evaluating to detect disease and bring patients to treatment. The incidence of biliary atresia is between one in 10 000 and one in 20 000 infants,8 while the incidence of all causes of neonatal liver disease presenting with jaundice is at least four times as high,9 and is sufficient to justify selective screening. In our opinion there have been sufficient observations on the beneficial effects of early detection to support the need for an early case finding programme for some years. Biliary atresia, a condition of unknown but probably diverse cause,10 is characterised by a progressive sclerosing cholangiopathy which causes complete obstruction of the extrahepatic bile ducts and early death from biliary cirrhosis. Many experienced centres with an expert surgeon have demonstrated that surgery after 60 days of age reduces the serum bilirubin to normal concentrations in only 20-35% of infants; with surgery before 60 days over 80% became jaundice free. l-14 One group has achieved even better results in those having surgery by 45 days of age.15 By 1991, it had been documented that the 15 year survival in infants who became jaundice free after surgical correction of biliary atresia was 87%. 16 Seventy per cent had no medical problems and were leading entirely normal lives. Without successful surgery infants with biliary atresia die at a mean age of approximately 1 year (a few survive until 7 years) unless treated with liver transplantation. With one year survival rates after one or more transplantation procedures approaching 90%,17 liver transplantation is a mode of treatment currently offered by the NHS for children in whom portoenterostomy has been unsuccessful. Liver transplantation requires many more resources than portoenterostomy and has more medical and surgical complications. 17-19 The patient needs lifelong immunosuppressive treatment. Thus although biliary atresia is the most common indication for liver transplantation in childhood, its role in biliary atresia is secondary to that of portoenterostomy. 19 Despite intensive professional education in the last 15 years8 11-13 20-23 over 500/0 of children with biliary atresia are referred after 6 weeks of age.8 Even now half still come to surgery too late for optimum results.24 To promote earlier recognition a number of recommendations were made,8 which were included in a list of desirable standards of care in the 1993 British Paediatric Association (BPA) publication on medical audit. These included the need to test for conjugated hyperbilirubinaemia in all infants who remain even mildly jaundiced after 14 days of age, the latter being supported by two subsequent reviews.25 26 At 15 days of age the infant with biliary atresia has mild jaundice but is likely to be otherwise well and the sclerosing process is Department of Child Health, Variety Club Children's Hospital, King's College Hospital, Denmark Hill, London SE5 9RS A P Mowat