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12 - Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts

from SECTION II - CHOLESTATIC LIVER DISEASES

Published online by Cambridge University Press:  18 December 2009

By
William F. Balistreri M.D. ,
Jorge A. Bezerra M.D.  and
Ryckman C. Frederick M.D.
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
William F. Balistreri M.D.
Affiliation:
Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Jorge A. Bezerra M.D.
Affiliation:
Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pediatric Gastroenterology, Hepatology, and Nutrition and the Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Ryckman C. Frederick M.D.
Affiliation:
Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Frederick J. Suchy
Affiliation:
Mount Sinai School of Medicine, New York
Ronald J. Sokol
Affiliation:
University of Colorado, Denver
William F. Balistreri
Affiliation:
University of Cincinnati
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Summary

Biliary atresia and related disorders of the biliary tract, such as choledochal cysts, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in the newborn (neonatal cholestasis). In this chapter, we review the current status of diagnosis and management of these disorders, as well as advances in the intriguing quest for an understanding of their pathogenesis.

OVERVIEW

Neonatal hepatobiliary diseases, including biliary atresia, choledochal cysts, and “idiopathic” neonatal hepatitis, have historically been viewed as a continuum – a gradation of manifestations of a basic underlying disease process in which giant cell transformation of hepatocytes is strongly associated with inflammation at any level of the hepatobiliary tract. These disease entities may be polar end points of a common initial insult, as originally stated in the unifying hypothesis of Landing [1]. The end result represents the sequela of the inflammatory process at the primary site of injury. Landing suggested that this inflammatory process may injure bile duct epithelial cells, leading to either duct obliteration (biliary atresia) or weakening of the bile duct wall with subsequent dilatation (choledochal cyst). The lesions may be dependent on the stage of fetal development when the injury occurs and the site within the developing hepatobiliary tree at which the injury occurs [1, 2]. A relationship of the pathogenesis of these obstructive cholangiopathies of infancy to the process of development is suggested by the association with disorders of situs determination such as the polysplenia syndrome and the observation of the so-called ductal plate malformation within the liver of a few patients with biliary atresia.

Type
Chapter
Information
Liver Disease in Children , pp. 247 - 269
Publisher: Cambridge University Press
Print publication year: 2007

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  • Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts
    • By William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Jorge A. Bezerra, M.D., Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pediatric Gastroenterology, Hepatology, and Nutrition and the Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Ryckman C. Frederick, M.D., Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.014
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  • Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts
    • By William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Jorge A. Bezerra, M.D., Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pediatric Gastroenterology, Hepatology, and Nutrition and the Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Ryckman C. Frederick, M.D., Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.014
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts
    • By William F. Balistreri, M.D., Dorothy M. M. Kersten Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Director, Pediatric Liver Care Center, Department of Pediatric Gastroenterolgy, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Jorge A. Bezerra, M.D., Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pediatric Gastroenterology, Hepatology, and Nutrition and the Pediatric Liver Care Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, Ryckman C. Frederick, M.D., Professor, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, Ohio; Surgical Director, Liver Transplant Program, Department of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
  • Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
  • Book: Liver Disease in Children
  • Online publication: 18 December 2009
  • Chapter DOI: https://doi.org/10.1017/CBO9780511547409.014
Available formats
×