Practice Easily Missed?

Biliary atresia

BMJ 2010; 340 doi: (Published 19 May 2010) Cite this as: BMJ 2010;340:c2383
  1. Jane Hartley, consultant paediatric hepatologist1,
  2. Anthony Harnden, university lecturer2,
  3. Deirdre Kelly, consultant paediatric hepatologist1
  1. 1Birmingham Children’s Hospital, Birmingham B4 6NH
  2. 2Department of Primary Health Care, Oxford University, Oxford OX3 7LF
  1. Correspondence to: J Hartley Jane.Hartley{at}
  • Accepted 22 April 2010

Biliary atresia is a rare (one in 17 000 in the United Kingdom1) but serious liver disorder that presents with jaundice in the first few weeks of life in apparently well infants. About 50 cases of biliary atresia occur each year in term babies who are born healthy and have usually had normal antenatal scans.2 The lumen of the biliary tree is obliterated by an inflammatory cholangiopathy, which obstructs the flow of bile from the liver to the intestine, resulting in progressive liver damage. Early surgery to reconstruct the biliary tree may reduce further damage and prevent the need for liver transplantation.

Case scenario

A mother presents to her general practitioner with her 3 week old son with mild jaundice; he is her first baby and fully breastfed. The mother was reassured. At the examination at age 8 weeks, the GP notices he has mildly icteric sclera and, on questioning, the mother states that his stools are cream coloured and his urine very yellow. The GP immediately refers the infant to the local paediatric unit, where further testing shows conjugated bilirubin concentrations of 120 μmol/l and leads to a final diagnosis of biliary atresia. The Kasai portoenterostomy carried out at age 9 weeks was unsuccessful, and he had a liver transplant at age 6 months.

How common is it?

  • Biliary atresia is the most common indication for liver transplantation in childhood

  • The younger the baby at the time of a Kasai portoenterostomy, the more likely the procedure is to be successful and avoid the need for liver transplantation

  • When the diagnosis of biliary atresia is made very late (beyond 100 days of age) children usually need a liver transplant in the first year of life

Why is it missed?

The diagnosis of neonatal liver disease, including biliary …

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