Clinical significance of liver histology on outcomes in biliary atresia

<jats:sec><jats:title>Aim</jats:title><jats:p>Biliary atresia (<jats:styled-content style="fixed-case">BA</jats:styled-content>) literature has focussed on the relationship between age at Kasai procedure (<jats:styled-content style="fixed-case">KP</jats:styled-content>) and post‐<jats:styled-content style="fixed-case">KP</jats:styled-content> outcomes. This study primarily examines post‐<jats:styled-content style="fixed-case">KP</jats:styled-content> outcomes including, 6‐month normalisation of bilirubin, 5‐year native liver survival (<jats:styled-content style="fixed-case">NLS</jats:styled-content>), development of portal hypertension (<jats:styled-content style="fixed-case">PHT</jats:styled-content>) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>Retrospective chart review of all <jats:styled-content style="fixed-case">BA</jats:styled-content> cases between 1999 and 2014. Age at <jats:styled-content style="fixed-case">KP</jats:styled-content>, liver biopsy results, use of ursodeoxycholic acid or prophylactic antibiotics and occurrence of <jats:styled-content style="fixed-case">PHT</jats:styled-content> and ascending cholangitis post‐<jats:styled-content style="fixed-case">KP</jats:styled-content> were recorded and related to the primary post‐<jats:styled-content style="fixed-case">KP</jats:styled-content> outcome measures.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> <jats:styled-content style="fixed-case">BA</jats:styled-content> was diagnosed in 29 patients. Twenty‐four of 29 patients underwent <jats:styled-content style="fixed-case">KP</jats:styled-content>. Median age at <jats:styled-content style="fixed-case">KP</jats:styled-content> was 68 days (29–104). Fourteen of 24 (58.3%) had bridging fibrosis and 5 of 24 (20.8%) had cirrhosis at time of <jats:styled-content style="fixed-case">KP</jats:styled-content>. Median follow‐up was 8.4 years (2.08–15.58 years). Bilirubin normalisation within 6 months occurred in 7 of 24 (29.2%) patients and 5‐year <jats:styled-content style="fixed-case">NLS</jats:styled-content> was 45.8% (11/24). Fourteen of 24 (58.3%) had <jats:styled-content style="fixed-case">PHT</jats:styled-content> and 18 of 24 (75%) patients had ascending cholangitis post‐<jats:styled-content style="fixed-case">KP</jats:styled-content>. Absence of bridging fibrosis in liver histology at <jats:styled-content style="fixed-case">KP</jats:styled-content> was the only factor to be significantly associated with improved 5‐year <jats:styled-content style="fixed-case">NLS</jats:styled-content>. None of the other variables examined had a significant association with either 5‐year <jats:styled-content style="fixed-case">NLS</jats:styled-content> or bilirubin normalisation by 6 months.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Five‐year <jats:styled-content style="fixed-case">NLS</jats:styled-content> in this series was 45.8%. Absence of bridging fibrosis at time of <jats:styled-content style="fixed-case">KP</jats:styled-content> was the only factor significantly associated with improved 5‐year <jats:styled-content style="fixed-case">NLS</jats:styled-content>.</jats:p></jats:sec>