Biliary atresia

Last reviewed: 21 Apr 2022
Last updated: 28 Oct 2020

Summary

Definition

History and exam

Key diagnostic factors

  • neonatal jaundice
  • acholic stool
More key diagnostic factors

Other diagnostic factors

  • dark urine
  • bruising
  • hepatomegaly
  • ascites
Other diagnostic factors

Risk factors

  • genetic predisposition
  • viral infection
  • environmental or behavioral exposures
  • immune dysregulation
More risk factors

Diagnostic investigations

1st investigations to order

  • serum total and direct or conjugated bilirubin
  • newborn screen (includes tests for galactosemia, thyroid dysfunction, cystic fibrosis, and a variety of metabolic diseases)
  • prothrombin time (PT), INR
  • CBC with differential
  • serum AST, ALT, ALP, and gamma-GT
  • abdominal ultrasound
More 1st investigations to order

Investigations to consider

  • hepatobiliary scintigraphy (technetium Tc 99m-di-isopropyl-acetanilido-imino-diacetic acid scan)
  • liver biopsy
  • cholangiogram
  • ERCP
  • CXR
  • infection screen: blood and urine cultures
  • urine PCR for cytomegalovirus
  • plasma or serum amino acids
  • alpha-1 level and protease inhibitor (Pi) type
  • random serum cortisol
  • urinary organic acids
  • urinary succinylacetone
  • urinary bile acids
  • serum lactate/pyruvate ratio
More investigations to consider

Treatment algorithm

ACUTE

infants with biliary obstruction without end-stage liver disease

infants with biliary obstruction with end-stage liver disease

ONGOING

post hepatoportoenterostomy

Contributors

Authors

Jessi Erlichman, MPH

Administrative Director

GI Clinical Research

The Children's Hospital of Philadelphia

Philadelphia

PA

Disclosures

JE receives payment for contributions to UpToDate.

Kathleen Loomes, MD

Professor of Pediatrics

Department of Pediatrics

The Children's Hospital of Philadelphia

Philadelphia

PA

Disclosures

KL receives payment for contributions to UpToDate and Wolters Kluwer. KL is a consultant for Albireo Pharmaceuticals and Mirum Pharmaceuticals.

Acknowledgements

Dr Jessi Erlichman and Dr Kathleen Loomes would like to gratefully acknowledge Dr Jonathan A. Flick and Dr Barbara A. Haber, previous contributors to this topic.

Disclosures

JAF and BAH declare that they have no competing interests.

Peer reviewers

Benjamin L. Shneider, MD

Director

Pediatric Hepatology Center

Visiting Professor of Pediatrics

University of Pittsburgh

Pediatric Hepatology Center

Pittsburgh

PA

Disclosures

BLS has received research funding from, and been a consultant to, several pharmaceutical companies that make antibiotic agents that might be used for treating bacterial prostatitis.

Mark D. Stringer, MD

Former Professor of Paediatric Surgery

University of Otago

Dunedin

New Zealand

Disclosures

MDS declares that he has no competing interests.

  • Biliary atresia images
  • Differentials

    • Extrahepatic biliary obstruction (e.g., choledochal cyst, spontaneous perforation of common bile duct, bile duct stricture or tumor, neonatal sclerosing cholangitis)
    • Hepatic viral infections (e.g., CMV, enterovirus, HSV, echovirus, adenovirus, hepatitis B virus, HIV, rubella, reovirus type 3, parvovirus B19, EBV)
    • Alagille syndrome
    More Differentials
  • Guidelines

    • Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of NASPGHAN and ESPGHAN
    • Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline
    More Guidelines
  • Patient leaflets

    Jaundice in newborn babies

    More Patient leaflets
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