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Congenital Biliary Dilatation

  • Atsuyuki YamatakaEmail author
  • Geoffrey J. Lane
  • Joel Cazares
Living reference work entry
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Abstract

Congenital biliary dilatation (CBD) continues to baffle researchers and surgeons because its cause remains unknown. There is a female preponderance and higher incidence in Asia. It is often identified in childhood but some 20% may remain undiagnosed until adulthood. Prenatal diagnosis has increased in recent years accounting for 25% of new cases. Hypotheses for its etiology and pathogenesis include an anomalous junction between the pancreatic duct and the common bile duct, intrahepatic bile duct dilatation, and hepatic fibrosis. Because there is a risk for malignancy increasing with age and inadequate surgery, early excision with Roux-en-Y hepaticoenterostomy is recommended. Intraoperative endoscopy should be performed routinely to prevent postoperative bile stone formation by washing out any debris present. Early and late complications include leakage, cholangitis, anastomotic stricture, and cholangiocarcinoma. Laparoscopic treatment is safe with outcomes comparable to open surgery.

Keywords

Choledochal cyst Hepaticojejunostomy Hepaticoduodenostomy Laparoscopic surgery Cholangiocarcinoma Pancreaticobiliary maljunction 

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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  • Atsuyuki Yamataka
    • 1
    Email author
  • Geoffrey J. Lane
    • 1
  • Joel Cazares
    • 1
    • 2
  1. 1.Department of Pediatric General and Urogenital SurgeryJuntendo University School of MedicineTokyoJapan
  2. 2.Department of Pediatric SurgeryHospital Regional de Alta Especialidad Materno InfantilMonterreyMexico

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