Elsevier

The Lancet

Volume 363, Issue 9418, 24 April 2004, Pages 1354-1357
The Lancet

Articles
Seamless management of biliary atresia in England and Wales (1999–2002)

https://doi.org/10.1016/S0140-6736(04)16045-5Get rights and content

Summary

Background

Before 1999, infants born in the UK with suspected biliary atresia were investigated in regional centres, and, if confirmed, a Kasai operation was done there. Since 1999, all infants with suspected biliary atresia in England and Wales, UK, have been referred to one of three designated centres where both the Kasai operation and liver transplantation (if necessary) could be done.

Methods

We assessed clearance of jaundice (bilirubin <20 μmol/L) as an early outcome in all cases of biliary atresia referred from one of the three centres. We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation or death.

Findings

148 infants with biliary atresia were treated between January, 1999, and June, 2002. A primary portoenterostomy was done in 142 (96%) infants and a primary liver transplant in five (3%). One child died before any intervention. Early clearance of jaundice after portoenterostomy was achieved in 81 of 142 (57%) infants. Liver transplantation was done in 52 (37%) of those undergoing portoenterostomy. 13 (9%) infants died. Of the 135 children who survived, 84 (62%) still have their native liver and 51 (38%) had transplantation. The median follow-up of survivors was 2·13 (range 0·5–4·1) years. The overall 4-year estimated actuarial survival was 89% (95% CI 82–94). The 4-year estimated actuarial survival with native liver was 51% (42–59%).

Interpretation

Our early results suggest that surgical outcome can be improved by centralisation of care to supra-regional centres.

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.

Section snippets

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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