Biliary Atresia is progressive inflammatory fibrosis of the biliary tract and the disease starts in the extra-hepatic bile ducts and extends into the intra-hepatic 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.

Click to view Medical Centric video on Biliary Atresia

Symptoms

Prolonged jaundice can be normal in breastfed babies however if there are signs of obstructive jaundice in a neonate, alarm bells should ring.  Infants with biliary atresia typically develop jaundice by 3 to 6 weeks of age.  Signs of obstructive jaundice include;

• yellowing of the skin
• yellowing of the whites of the eyes (sclera)
• pale coloured stools
• dark coloured urine

Diagnostic Tests

• Blood tests shows abnormal liver function tests with conjugated fraction of the bilirubin more than 20% of total serum bilirubin
• Liver ultrasound scan may show fibrosis of the extra-hepatic biliary tree
• Liver biopsy will show inflammation and scarring of the liver cells
• Cholangiogram (a dye injected into the gall bladder with an X-ray to see the flow of the dye in the liver) will usually give the definitive diagnosis

Treatment

Surgical management for biliary atresia is the Kasai porto-enterotomy procedure (named after Dr. Morio Kasai, the Japanese surgeon who developed it).  The aim of the procedure is 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.  In infants who respond well, the jaundice should improve within a few weeks.

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.  In cases where the Kasai procedure does not work, the problem often lies in the fact that the blocked bile ducts are within the liver (known as intrahepatic ducts).  These infants unfortunately, may still need a liver transplant later on in life.

Post-Treatment Care

In addition to surgery, the infant will need 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

See a Paediatric Surgeon to discuss your treatment options

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