Biliary Atresia

Biliary Atresia is progressive inflammatory fibrosis of the biliary tract and the disease starts in the extrahepatic bile ducts and extends into the intrahepatic ducts. Liver cells produce bile to help with fat digestion in the intestine.

When bile cannot flow into the intestine, the bile builds up in the liver causing liver damage resulting in scarring and cirrhosis.

The incidence in Asia is around 1 in 10,000 – 20,000 live births. Some infants (around 10-15%) with biliary atresia have other congenital problems such as spleen abnormality (polysplenia), blood vessel anomalies or intestine mal-rotation.

Symptoms

Prolonged jaundice can be normal in breastfed babies however if there are signs of obstructive jaundice in a neonate, alarm bells should ring.

Signs of obstructive jaundice are yellowing of the skin and whites of the eyes (sclera), pale coloured stools with dark coloured urine in an otherwise normal child. Infants with biliary atresia typically develop jaundice by 3 to 6 weeks of age.

An infant with jaundice and pale stools should be referred to a paediatric surgeon urgently. The serum liver function test is usually abnormal.

Typically, with biliary atresia, the conjugated fraction of serum bilirubin is more than 20% of the total serum bilirubin. (although the bilirubin total may not be extremely high).

Tests

An ultrasound scan may show fibrosis of the extrahepatic biliary tree and liver biopsy.  A cholangiogram (a dye injected into the gall bladder with an X-ray to see the flow of the dye in the liver) will give the definitive diagnosis.

Treatment

Surgical management for biliary atresia is the Kasai porto-enterotomy procedure to re-establish bile flow out of the liver and into the intestine.  The outcome for biliary atresia surgery is correlated to the timing of surgery. The earlier the procedure (ideally before 60 days), the better the rate for survival of the child’s own liver.

The success of the procedure also depends on how damaged the liver is at the time of surgery and the number of viable ducts left in the liver to drain the bile. Some infants unfortunately, may still need a liver transplant later on in life.

In addition to surgery, the infant needs careful supervision of daily diet to include:

  • Well balanced diet
  • Vitamin supplements
  • Addition of medium chain triglyceride (MCT) oil to the diet to add extra calories
  • High-calorie liquid feeding if the infant is too ill to eat normally

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