Kat Ford1, Maria Asimakidou1, Jessica Burns1, Andrew Page1, Michael Dawrant2, Naved Alizai2, Khalid Sharif3, Evelyn Ong3, Erica Makin1, Mark Davenport1
1Kings College Hospital, London, United Kingdom. 2Leeds General Infirmary, London, United Kingdom. 3Birmingham Childrens Hospital, Birmingham, United Kingdom


Aims: Biliary atresia (BA) is rare with significant variation in incidence across the globe. National centralisation of management since 1999 has allowed collation of a unique dataset to allow study of its background.

Methods: Prospective national dataset since 1999 of all infants born with BA in England and Wales. BA was divided into two groups based on presumed aetiology [isolated biliary atresia (IBA) and developmental biliary atresia (DBA) (I.E. Other anomalies+BA; cystic biliary atresia). Incidence was compared across the period and health region using appropriate statistical tests. A P value of 0.05 was considered significant.

Results: There were 703 (54% female)) infants with BA that could be divided into IBA (n = 516, 73.3%) and DBA (n = 187, 26.6%). The overall incidence was 0.62/10 000 (1 in 16,169) live births with a marked regional difference varying from 0.44 (north-west England) to 0.68 (London, south-east England, eastern England)/10 000 live births (P < 0.001). Relative risk in north-west (vs London)=0.64 (95%CI 0.47 – 0.86) P= 0.003). This difference was due to a relative reduction in incidence of IBA in the north-west rather than DBA and was associated with the highest proportion of infants from a Caucasian parental background (86.9%)

502/682 (73.6%) were from a Caucasian parental background and overall there was no difference in relative distribution of IBA and DBA (P=0.20), except for West Midlands (IBA 72% (Cauc.) vs 89% (Non-Cauc.) (P = 0.02).

The number of cases/year varied from 28 to 59 though there was no overall trend across the period (P = 0.08).  The commonest month of birth was August although there was no evidence for significant seasonal variation (P = 0.22). 


  • Remarkable variation of incidence of biliary atresia within England and Wales due to variation in those with isolated BA.