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Biliary atresia

Biliary atresia (BA) is a congenital biliary disorder, which is characterised by an absence or severe deficiency of the extra-hepatic biliary tree. It is one of the most common causes of neonatal cholestasis, often causing cirrhosis immediately and leading to death and accounts for over half of children who undergo liver transplantation. 


It is thought to affect 1 in 10,000-15,000 newborn infants. There is a recognised male predilection.

Clinical presentation

 It precipitates within the first three months of life. Infants with biliary atresia may appear normal and healthy at birth. Most often, symptoms develop between two weeks to two months of life, and may include :

  • jaundice (conjugated hyperbilirubinaemia)
  • dark yellow or brown urine
  • pale or clay-colored (acholic) stools
  • hepatomegaly



It is thought to result from idiopathic destructive inflammatory process which leads to fibrotic remnants at porta hepatis.


In 10% of cases, there is an association with polysplenia and heterotaxy syndrome 6.


Kasai classification is used to classify the three main anatomical types of biliary atresia.

Radiographic features

Prompt diagnosis ensures early treatment and results in improved prognosis.

Nuclear medicine (hepatobiliary (HIDA) scan)

Tc-99m diosgenin (DISIDA) and mebrofenin (BRIDA) have highest hepatic extraction rate and shortest transit time of hepatobiliary radiotracers. Cases of biliary atresia typically demonstrate relatively good hepatic uptake with no evidence of excretion into the bowel at 24 hours. Pretreatment with phenobarbital (5 mg/kg/day for 5 days) to increase biliary secretion by stimulating hepatic enzymes is frequently helpful to minimize the possibility of a false-positive study in a patient with a patent biliary system but poor excretion.

Treatment and prognosis

Management options include


Differential diagnosis

General imaging differential considerations include

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