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Biliary Atresia

  • Dan W. Parrish
  • Jonathan H. DeAntonio
  • David A. LanningEmail author
Chapter

Abstract

A newborn with greater than 2 weeks of jaundice or a primarily conjugated hyperbilirubinemia should be evaluated thoroughly for a cause. Typical symptoms of biliary atresia are elevated conjugated bilirubin, jaundice, acholic stools, and possibly hepatomegaly. There is no definitive test to diagnose biliary atresia; the workup consists of ruling out other diseases and, typically, a HIDA scan and right upper quadrant ultrasound. An intraoperative cholangiogram can be used to demonstrate the biliary anatomy and rule out biliary atresia. A liver biopsy is also performed. The operation of choice for biliary atresia is the Roux-en-Y hepatic portoenterostomy, Kasai procedure. Outcomes include successful long-term biliary drainage (33%), temporary drainage but will be older and more able to withstand a liver transplant (33%), and no relief with progression to liver failure requiring transplantation (33%).

Keywords

Biliary atresia Jaundice Conjugated hyperbilirubinemia Acholic stools Rou-en-Y hepatic portoenterostomy Kasai procedure 

References

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Dan W. Parrish
    • 1
  • Jonathan H. DeAntonio
    • 2
    • 3
  • David A. Lanning
    • 2
    • 3
    • 4
    Email author
  1. 1.Department of Pediatric SurgeryBatson Children’s Hospital, University of Mississippi Medical CenterJacksonUSA
  2. 2.Division of Pediatric Surgery, Department of General SurgeryVirginia Commonwealth University HealthRichmondUSA
  3. 3.Department of SurgeryVirginia Commonwealth University School of MedicineRichmondUSA
  4. 4.Department of Surgery and PediatricsChildren’s Hospital of RichmondRichmondUSA

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