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Biliary Atresia and Other Congenital Disorders of the Extrahepatic Biliary Tree

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Pediatric Hepatology and Liver Transplantation

Abstract

Biliary atresia (BA) and congenital choledochal malformation (CCM) are the major surgical causes of obstructive jaundice in infancy with spontaneous bile duct perforation (SBDP) an important minor cause.

BA is characterized by obliteration of both intra- and extrahepatic bile ducts and presents as conjugated jaundice and pale stools in the first weeks of life. It is a condition seemingly unique to the neonatal period. Treatment is entirely surgical being an attempt to restore bile flow from the native liver in the first instance (Kasai portoenterostomy), reserving liver transplantation for those where this approach is unsuccessful. CCM presenting during infancy is usually cystic in nature and frequently associated with an anatomically abnormal union between the pancreatic and common bile ducts. A proportion will present with an abnormal maternal ultrasound scan, but some may become truly obstructed during the first few weeks of life. Later examples of CCM (both fusiform and cystic) may cause abdominal pain (typically pancreatitis) and/or obstructive jaundice at any age. Radical cyst excision and hepaticojejunostomy achieve good results in infants and older children. SBDP is the least common cause of surgical jaundice and “ascites” in infants. Sometimes there is an underlying reason such as inspissation of bile or choledochal malformation, but many are simply idiopathic.

M.D. dedicates this work as always to Georgina.

P.B. dedicates this work to Valentina, Paolo and Bianca.

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  1. Department of Paediatric Surgery, Papa Giovanni XXIII Hospital, Bergamo, Italy

    Pietro Betalli

  2. Department of Paediatric Surgery, King’s College Hospital, London, UK

    Mark Davenport

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  1. Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy

    Lorenzo D'Antiga

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Betalli, P., Davenport, M. (2019). Biliary Atresia and Other Congenital Disorders of the Extrahepatic Biliary Tree. In: D'Antiga, L. (eds) Pediatric Hepatology and Liver Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-319-96400-3_7

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