ArticlesSeamless management of biliary atresia in England and Wales (1999–2002)
Introduction
Biliary atresia is an obliterative cholangiopathy of unknown cause with an incidence in western Europe of about one in 12 000–18 000 infants, which implies that some 40–45 affected infants are born every year in England and Wales, UK. Management of these children is mainly surgical, with, typically, an initial attempt to restore bile flow by excision of all extrahepatic biliary remnants and reconstruction with a portoenterostomy to a jejunal loop (the Kasai operation). For infants in whom clearance of jaundice is inadequate, or complications of chronic liver disease supervene, or in whom initial biliary surgery is thought to be futile (because of decompensated cirrhosis for instance), then liver transplantation is indicated.
A survey1 of practice and outcome of all infants born with biliary atresia in the UK and Ireland between 1993 and 1995 by the British Paediatric Surveillance Unit showed that 15 paediatric surgical centres were doing the Kasai operation. Outcomes differed significantly when centres were divided into groups depending on caseload (more than five cases per year versus fewer than five cases per year) although some elements of the interpretation were controversial and disputed.2, 3 These differences led, in part, to a UK Department of Health directive for England and Wales (DoH 199/0268 30.4.99) indicating that all infants with suspected biliary atresia should be referred to one of three designated centres where both the Kasai operation and, if necessary, liver transplantation could be offered. Here, we aimed to assess the early outcomes resulting from this change in policy.
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Cases
We identified all cases of biliary atresia referred from centres within England and Wales to one of the three designated centres between January, 1999, and June, 2002. The three centres were King's College Hospital, London (Centre A); Birmingham Children's Hospital (Centre B), and St James' University Hospital, Leeds (Centre C). To our knowledge, no infant underwent any form of surgery for biliary atresia outside these centres in this period.
Macroscopic classification of biliary atresia was
Results
148 infants with biliary atresia were referred to the three centres: 74 to centre A, 54 to centre B, and 20 to centre C. One infant, referred at 13 weeks of age with biliary atresia splenic malformation syndrome and congenital heart disease (pulmonary and subaortic stenosis), initially received a pulmonary angioplasty, but died of cardiac complications. A primary Kasai portoenterostomy was done in 142 infants (median age at surgery 54 [range 7–175] days) and a primary liver transplantation in
Discussion
The expectation of survival of infants born with biliary atresia has reversed over the past 30 years. For example, of 11 infants born with biliary atresia in one English region during 1971–73, only one child survived to 10 years of age—the remainder died within their first 2 years.5 At present in England and Wales, about 90% of children with this disorder are surviving, at least in the short-term, through use of a complementary surgical programme of Kasai portoenterostomy and liver
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