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Atresia das vias biliares

  • Visão geral
  • Teoria
  • Diagnóstico
  • Tratamento
  • ACOMPANHAMENTO
  • Recursos
Última revisão: 16 Aug 2025
Última atualização: 12 Sep 2023

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • icterícia neonatal
  • fezes acólicas
Detalhes completos

Outros fatores diagnósticos

  • urina escura
  • hematomas
  • hepatomegalia
  • ascite
Detalhes completos

Fatores de risco

  • predisposição genética
  • infecção viral
  • exposições ambientais ou comportamentais
Detalhes completos

Diagnostic investigations

1st investigations to order

  • bilirrubina sérica total e direta ou conjugada
  • rastreamento do neonato (inclui exames para galactosemia, disfunção tireoidiana, fibrose cística e uma variedade de doenças metabólicas)
  • tempo de protrombina (TP), razão normalizada internacional (INR)
  • Hemograma completo com diferencial
  • aspartato transaminase (AST), alanina aminotransferase (ALT), fosfatase alcalina (FAL) e gama-glutamiltransferase séricas
  • ultrassonografia abdominal
Full details

Investigations to consider

  • cintilografia hepatobiliar (cintilografia com ácido iminodiacético-acetanilida-diisopropil tecnécio-99m)
  • biópsia hepática
  • colangiografia
  • radiografia torácica
  • rastreamento de infecção: culturas de sangue e urina
  • reação em cadeia da polimerase da urina para citomegalovírus
  • aminoácidos plasmáticos ou séricos
  • nível de alfa-1 e tipo de inibidor da protease (Pi)
  • cortisol sérico aleatório
  • ácidos orgânicos urinários
  • succinilacetona urinária
  • ácidos biliares urinários
  • razão lactato sérico/piruvato
Full details

Treatment algorithm

ACUTE

lactentes com obstrução biliar sem doença hepática em estágio terminal

lactentes com obstrução biliar com doença hepática em estágio terminal

ONGOING

pós-hepatoportoenterostomia

Contributors

Authors

Jessi Erlichman, MPH

Senior Director

Research Administration

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. KL is on an advisory panel for Albireo Pharmaceuticals and Mirum Pharmaceuticals and is a consultant for Travere Therapeutics.

Acknowledgements

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

Professor of Pediatrics

Service Chief in Pediatric Gastroenterology, Hepatology and Nutrition

Baylor College of Medicine

Texas Children’s Hospital

Houston

TX

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.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Fawaz R, Baumann U, Ekong U, et al. Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2017 Jan;64(1):154-68.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Atresia das vias biliares images

  • Differentials

    • Obstrução biliar extra-hepática (por exemplo, cisto de colédoco, perfuração espontânea do ducto colédoco, estenose ou tumor do ducto biliar, colangite esclerosante neonatal)
    • Infecções virais hepáticas (por exemplo, citomegalovírus [CMV], enterovírus, vírus do herpes simples [HSV], ecovírus, adenovírus, vírus da hepatite B, vírus da imunodeficiência humana [HIV], rubéola, reovírus do tipo 3, parvovírus B19, vírus Epstein-Barr [EBV])
    • Síndrome de Alagille
    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 information

    Icterícia em neonatos: quais tratamentos funcionam?

    Icterícia em neonatos: o que é?

    More Patient information
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