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).