Biliary Atresia

Biliary atresia is a condition of the bile duct that is not formed or does not develop normally. Bile removes metabolic waste from the liver and transports the salt needed to digest fat in the small intestine.

In biliary atresia there is a blockage of bile flow from the liver to the gall bladder. This can cause liver damage and cirrhosis of the liver which if left untreated can be fatal.

Cause. 

• Biliary atresia occurs due to abnormal development of the bile ducts inside and outside the liver. But the cause of this development of bile ducts is unknown.
• Biliary atresia is found in 1 in 15,000 births.

Symptoms. 

Symptoms usually occur within 2 weeks after birth, which are in the form of :

• Dark baby urine.
• Pale stools.
• Yellow skin.
• Weight does not increase or weight gain takes place slowly.
• Heart enlarged.

When the baby reaches 2-3 months, the following symptoms will occur :

• Growth disturbance.
• Itchy.
• Fussy.
• High blood pressure in the portal vein (blood vessels that carry blood from the stomach, intestine and spleen to the liver). 

Diagnosis. 

• Diagnosis is based on symptoms and physical examination.
• On an abdominal examination, the palpable heart enlarges.

Checks are usually done :

• Blood tests (there is an increase in bilirubin levels).
• Stomach ultrasound.
• X-ray of the abdomen (looks enlarged heart).
• Colangiogram.
• Liver biopsy.
• Laparotomy (usually done before the baby is 2 months old).

Treatment. 

• The best procedure is to replace the bile ducts that drain bile into the intestines. But this procedure is only possible in 5-10% of sufferers.
• To bypass biliary atresia and directly connect the liver to the small intestine, a surgery called the Kasai procedure is performed.
• Surgery will be successful if done before the baby is 8 weeks old.
• Usually this surgery is only a temporary treatment and in the end it is necessary to have a liver transplant.