Epidemiology of Biliary Atresia in England and Wales (1999 - 2006)

Emily Livesey ¹, Mario Cortina Borja ², Khalid Sharif ³, Naved Kamal Alizai ⁴, Patricia McClean ⁴, Deidre A Kelly ³, Nedim Hadzic ¹ and Mark Davenport ^1*

¹ Kings College Hospital, United Kingdom
² Institute of Child Health, London, United Kingdom
³ Birmingham Children's Hospital, United Kingdom
⁴ St James' Hospital, Leeds, United Kingdom

^* To whom correspondence should be addressed. E-mail: markdav2{at}ntlworld.com.

Accepted 10 May 2009

Abstract

Objective: To identify the epidemiological characteristics of infants with biliary atresia in England and Wales, since centralisation of its management in 1999.

Methods: The care of infants with biliary atresia (BA) in England and Wales is centralized to only three centres. All infants (treated from Jan. 1999 to Dec. 2006) were identified from a prospective national database; demographic details were ascertained from medical records and compared between two groups based on presumed aetiology [isolated biliary atresia (IBA) and developmental biliary atresia (DBA) (e.g. syndromic infants, BASM, cystic biliary atresia)].

Results: There were 302 (133 male (44%)) infants with BA that could be divided into IBA (n = 219, 73%) and DBA (n = 76, 25%). The overall incidence was 0.58 / 10,000 (i.e. 1 in 17,049) live births with marked regional differences along a north-west / south –east axis varying from 0.38 (North-West England) to 0.78 (South-East England) /10,000 live births (OR 2.05 (95%CI 1.26 – 3.41); P =0.002). The commonest month of birth was September with December being the least common, although there was no evidence for significant seasonal variation (P = 0.2). Infants with DBA were more likely to be female (P < 0.001), of Caucasian background (P = 0.01), first-born (P = 0.04) and to be formula-fed (P = 0.07). Infants of South Asian origin came to surgery at an older age [59 (IQ 45-75) versus 52 (IQ 42-65); P = 0.03] days.

Conclusions: There is a remarkable variation of incidence of biliary atresia within England and Wales, some of which may have been caused by factors related to a different aetiological and racial background.