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Biliary Atresia

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Diseases of the Liver in Children

Abstract

Biliary atresia (BA) is a severe hepatobiliary disease in infancy characterized by a progressive, fibro-obliterative process affecting extrahepatic as well as intrahepatic bile ducts, leading to early liver cirrhosis. The etiology is unknown but is most likely multifactorial. Kasai hepatic portoenterostomy can provide effective palliation and successful bile drainage which correlates with the patient’s age at operation. Screening by stool color card is useful in early identification of BA from infants with cholestasis and timely portoenterostomy to restore bile drainage. Recent studies showed a clear disadvantage for those infants operated on later than 90 days of age. Postsurgical management includes prevention of cholangitis, nutritional support to overcome malabsorption and malnutrition, and minimization of the sequelae of portal hypertension. About 80 % of children with successful portoenterostomy can survive more than 10 years with their native liver and achieve good quality of life. However, a large proportion of children with BA will require liver transplantation at some stage, especially if the Kasai portoenterostomy is unsuccessful. In the largest follow-up study of BA children who underwent liver transplantation, the 10-year patient and actuarial graft survival was 86 % and 73 %, respectively. While the improved medical and surgical management and liver transplantation have prolonged survival, further investigation on the etiology and pathogenesis of BA will hopefully provide effective therapy to intervene the development and progression of the disease in the future.

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  1. Department of Pediatrics, National Taiwan University Hospital, No. 7, Chung-Shan South Road, Taipei, Taiwan

    Hong-Yuan Hsu MD, PhD & Mei-Hwei Chang MD

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  1. Hong-Yuan Hsu MD, PhD
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Correspondence to Mei-Hwei Chang MD .

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  1. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Karen F. Murray

  2. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Simon Horslen

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Hsu, HY., Chang, MH. (2014). Biliary Atresia. In: Murray, K., Horslen, S. (eds) Diseases of the Liver in Children. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9005-0_13

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  • DOI: https://doi.org/10.1007/978-1-4614-9005-0_13

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