PassFinals
Paediatrics

Biliary atresia

Biliary atresia is progressive obliteration of the extrahepatic bile ducts causing neonatal cholestasis; persistent jaundice with pale stools or dark urine needs prompt split bilirubin testing and urgent specialist referral because early surgery offers the best chance of preserving the native liver.

In a nutshell

Biliary atresia is a progressive cause of neonatal cholestasis. In a term baby jaundiced for more than 14 days, or a preterm baby jaundiced for more than 21 days, check total and conjugated bilirubin. Pale or chalky stools, dark urine and significant conjugated bilirubin require prompt specialist liver-centre discussion; do not wait for every test. The usual definitive operation is early Kasai hepatoportoenterostomy, with transplantation considered if drainage fails or liver disease progresses.

Classic presentation

A 3-week-old term baby remains jaundiced, has pale stools and dark urine, and has a raised conjugated bilirubin. The baby may still be feeding and growing well. The correct response is urgent specialist discussion, not reassurance or phototherapy.

Key points

  • Prolonged jaundice means more than 14 days in a term baby or more than 21 days in a preterm baby.
  • Measure total and conjugated bilirubin; conjugated bilirubin above 25 micromol/L or above 25% of total requires prompt investigation and specialist discussion.
  • Pale stools and dark urine are cholestasis red flags; a well-looking baby can still have biliary atresia.
  • Ultrasound can support the diagnosis or identify alternatives, but a normal scan does not exclude biliary atresia.
  • Kasai hepatoportoenterostomy is the usual first operation and is more likely to succeed when performed early; specialist follow-up remains essential.
  • Do not invent or memorise product doses for cholestasis: use the current BNF for Children and the specialist liver-centre protocol.

First-line investigation

Total and conjugated bilirubin in any term baby jaundiced for more than 14 days or preterm baby jaundiced for more than 21 days.

Management

Split the bilirubin

  • Check total and conjugated bilirubin after the NICE prolonged-jaundice time point and ask specifically about pale stools and dark urine.1,2

Discuss urgently

  • Significant conjugated bilirubin, pale stools or an unwell infant warrants prompt or urgent specialist liver-centre discussion; investigate treatable causes in parallel.1,2

Restore drainage early

  • The specialist team confirms the anatomy and arranges Kasai hepatoportoenterostomy as early as appropriate; earlier surgery is associated with a better chance of success.3

Support and monitor

  • Support nutrition, glucose, coagulation and vitamin status, then monitor drainage, growth, cholangitis and portal hypertension; assess for transplantation if liver disease progresses.2,4

Exam traps

  • Do not call prolonged neonatal jaundice physiological without checking the conjugated fraction.
  • Pale stools plus dark urine indicate cholestasis; do not use phototherapy as treatment for biliary obstruction.
  • A normal ultrasound or absent HIDA excretion alone does not prove or exclude biliary atresia.
  • Kasai is the usual first operation; transplantation is considered for failed drainage or progressive liver failure, not as the routine initial operation.
  • The 60-day timing statement describes improved likelihood of success, not a safe reason to delay referral or an absolute cut-off.

Illustrations

Operative appearance of extrahepatic biliary atresiaA sourced operative image illustrating extrahepatic biliary atresia; retain attribution and licence information and keep image-quality remediation in the separate image campaign.Chardot C, Wikimedia Commons · CC-BY-2.0

Key sources

  1. NICE CG98, Jaundice in newborn babies under 28 days (Recommendations on prolonged jaundice, stool and urine colour, conjugated bilirubin, blood tests and seeking expert advice; guideline last updated 31 October 2023)Updated 31 Oct 2023
  2. BSPGHAN Liver Steering Group, Guideline for the investigation of neonatal conjugated jaundice (UK paediatric hepatology guideline revised December 2016; recommends prompt investigation of significant conjugated jaundice and urgent specialist discussion for pale stools or an ill infant)Updated 1 Dec 2016
  3. UK National Screening Committee, consultation on screening for biliary atresia in newborns (Current UK NSC consultation information published 25 February 2026; explains the time-sensitive symptom-to-surgery pathway and that surgery is more likely to succeed before 60 days; consultation status must not be confused with a final screening recommendation)Updated 25 Feb 2026
  4. BNF for Children, current prescribing information (UK prescribing source for vitamin K and fat-soluble-vitamin preparations; direct product pages were restricted during this review, so no unsupported dose was added and dosing remains specialist/local-protocol led)

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.