A Tertiary Centre Experience in the Diagnosis of Biliary Atresia

Abstract

Biliary atresia (BA) is rare but is the most common cause of liver transplant in children. Early diagnosis and Kasai portoenterostomy (KP) improves outcomes. A retrospective chart review of all infants diagnosed with BA between January 1, 2002 and December 31, 2009 was conducted. The median age at referral, KP, and place of residence at presentation were determined. Age at which a conjugated bilirubin was first ordered was compared for various health care providers. Survival at 2 years of age with a native liver was determined. Seventeen patients were diagnosed with BA and 13 underwent a KP. Biliary atresia splenic malformation (BASM) was present in 23.5% of patients. The median age of referral to a tertiary centre was 56d (IQR 42; range: 19-118 d) and the median age at KP was 55d (IQR: 30; range 26-102 d), neither of which differed significantly from established Canadian medians. Paediatricians requested conjugated bilirubin level more frequently than family physicians; 58% vs 29%. Neither the rurality of patients’ residence nor the type of health care provider for well-baby care influenced age at referral to subspecialist. Two-year survival with native liver was 15.4%. There was no evidence of a difference in age at referral or age at KP observed in this study compared with established median for Canada. The place of residence didn’t influence timing of referral. The study noted a lower than expected survival with native liver at 2 years.