Biliary atresia

Biliary atresia (BA) is a congenital biliary disorder, which is characterised by an absence or severe deficiency of the extrahepatic 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. 

Epidemiology

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

Pathology

Aetiology

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

Associations

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

Classification

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.

Ultrasound
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

Complications

Differential diagnosis

General imaging differential considerations include

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rID: 5176
Tag: cases
Synonyms or Alternate Spellings:
  • Biliary atresia (BA)
  • Atresia of the bile ducts
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    Case 1: showing triangular cord sign
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