Congenital biliary atresia: liver injury begins at birth

• Published in: Journal of pediatric surgery 2009-03, Vol.44 (3), p.630-633
• Main Authors: Makin, Erica, Quaglia, Alberto, Kvist, Nina, Petersen, Bodil Laub, Portmann, Bernard, Davenport, Mark
• Format: Article
• Language: English
• Subjects: Biliary atresia; Biliary Atresia - blood; Biliary Atresia - pathology; Biliary Atresia - physiopathology; Biliary Atresia - surgery; Biliary atresia splenic malformation; Bilirubin - blood; Fatal Outcome; Female; Fibrosis; Humans; Liver - metabolism; Liver - pathology; Male; Neonatal cholestasis; Pediatrics; Retrospective Studies; Surgery
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2008.10.069

Abstract Background The timing of onset of liver injury in biliary atresia (BA) is not known, although in approximately 10% of cases, biliary pathologic condition associated with the biliary atresia splenic malformation syndrome must begin well before birth. Methods The study involved retrospective case-note review for infants with definite BA who underwent laparotomy within first week of life. Results Three infants were identified who had occlusive BA evident on the first day of life. In all cases, their liver was grossly normal, and histologic changes were trivial. Conclusion This suggests that the detrimental cholestatic liver injury, later characteristic of BA, only begins from the time of birth despite a prenatal occlusive biliary pathology. It may be that tissue injury only occurs with the onset of the perinatal bile surge initiating periductal bile leakage and the triggering of an inflammatory and ultimately fibrotic response.