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BILIARY ATRESIA AND ASSOCIATED STRUCTURAL ANOMALIES: CLINICAL AND OUTCOME FINDINGS CANADA-WIDE
OR Guttman1, RA Schreiber2, CC Barker2, VL Ng1, SC Ling1, EA Roberts1, and the Canadian Pediatric Hepatology Research Group
1Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario; 2Division of Gastroenterology, BC Children’s Hospital, Vancouver, British Columbia
INTRODUCTION: Although biliary atresia (BA) occurs as an isolated bile duct disease in a majority of affected infants, a distinct phenotypic subgroup exists in association with congenital abnormalities. Some studies have reported a poorer outcome for this group, although their phenotypic definition has varied substantially from study to study. This report aims to describe the Canadian experience with these patients.
METHODS: A retrospective review was undertaken of the Canada-wide database of BA patients born from 1985-2002 and followed at one of twelve tertiary-care hospitals. Patients with =>1 of the following anomalies were identified: a/polysplenia, abnormal abdominal situs, intestinal malrotation, abnormal pancreas, portal vein or hepatic artery anomaly, IVC interruption or congenital heart disease.
RESULTS: Among a total of 349 patients, 37 (19 girls) were found to have associated congenital anomalies. Associated structural anomalies included: polysplenia (n=18), abnormal abdominal situs (n=8), intestinal malrotation (n=15), portal vein anomaly (n=9), hepatic artery anomaly (n=1), IVC interruption (n=14). Congenital cardiac malformations were found in 23 patients, including VSD (n=8), left atrial isomerism (n=7), ASD (n=5), pulmonary stenosis (n=6), tetralogy of Fallot (n=3), total anomalous pulmonary venous return (n=2), AV canal (n=2), double outlet right ventricle (n=2), partial anomalous pulmonary venous return (n=1), dextrocardia (n=1), hypoplastic left heart (n=1), bicuspid aortic valve (n=1), bicuspid pulmonary valve (n=1) and mitral stenosis (n=1). Two patients had => 2 significant anomalies without associated polysplenia. Median age at Kasai was 67 (10-146) days. Overall survival was significantly worse at 4 years among patients with anomalies (65% vs. 79.6%)*. Post-Kasai survival with native liver at age 4 years was also poorer in the patients with anomalies (30% vs. 38%)*. *For the 33 patients with anomalies for whom data are available.
CONCLUSIONS: Four-year overall survival and 4-year survival with native liver are poorer among BA patients with associated structural anomalies.