Biliary Atresia

Biliary atresia (BA) is a serious disease that occurs worldwide and affects 1 in 8000 to 15000 newborn infants, causing interruption to bile flow that can lead to serious health problems.

The roles of bile

Bile is the liquid produced by the liver to aid digestion and absorption of fats and fatsoluble vitamins in the small intestine. Bile also helps to carry bilirubin from the liver to intestines for excretion.

Normal and healthy bile ducts – inside and outside the liver – allow bile to flow from the liver to the gallbladder for storage, before it is excreted into the intestine to carry out its functions.

However, when a baby has BA, the bile ducts are absent or damaged and blocked. Impaired bile flow leads to liver damage, affecting many vital body functions at the same time. It can be fatal if not treated.

The causes of BA are still unknown, but it is believed to be caused by multiple factors. What is known so far is that BA is not an inherited disease, and it rarely occurs more than once in the same family. It also has nothing to do with medications taken by women during pregnancy.

Types of BA
Postnatal BA This is the most common form of BA, which develops shortly after birth Newborns may appear healthy at birth, but the signs will become evident within the first few weeks after birth.	Foetal BA This type of BA is less common, and is due to improperly formed bile ducts when baby is still a foetus. Infants with foetal BA may also have other birth defects in the heart, spleen, blood vessels or intestines.

Signs to watch out

The very first sign of biliary atresia is jaundice (a yellow discolouration of the skin and the whites of the eyes). Other common signs of BA include pale-coloured stools and dark urine. In “postnatal” BA, the infants are usually born at term with an appropriate weight. On the other hand, infants with the “foetal” form of BA are more likely to be underweight. Parents should be aware that jaundice can also occur in many healthy newborns during the first few days of life. This harmless physiological jaundice usually clears within 2 weeks. In BA, however, infants will experience prolonged jaundice and have pale stools.

If BA is suspected, the infant is usually referred to a centre where there are paediatric specialists and paediatric surgeons with experience in diagnosing and treating the condition.

Treatment for BA

Biliary atresia is a condition that cannot be treated with medication. The only first-line treatment to improve bile flow and reduce jaundice is a surgical procedure called the Kasai portoenterostomy. Prior to the development of this procedure, death from BA was almost 100%.

Nutritional needs in BA

Nutrition can be a problem in a child with BA. Due to inadequate or absence of bile in the intestine, digestion and absorption of fats and certain vitamins can be significantly hampered. Children with BA also have faster metabolism than healthy children, so they require more calories.

If your child has BA, you can take these steps to ensure that his nutritional needs are not compromised:

• Seek the advice of your paediatrician and dietician.
• Feed him a well-balanced diet.
• Offer him three regular meals a day and light snacks in between meals.
• Supplement his diet with vitamins, eg vitamins A, D, E and K.
• Medium-chain triglyceride (MCT) oil may be added to his foods and drinks to give him more calories and help him grow. Compared to other types of fats, MCT are more easily digested without bile.

Kasai procedure Performed to create an open duct to reestablish bile flow from the liver to the intestine. Damaged ducts outside the liver are removed and replaced with a loop of the baby’s own intestine. This allows the bile to pass from the liver into the intestine. This procedure is not a cure for BA, but if bile continues to flow, long-term survival is possible.

Liver transplant This option needs to be considered when the Kasai procedure is not successful (ie still not enough bile flow). It is a well-accepted treatment for children with BA and end-stage liver disease. The damaged liver is removed and replaced with a new liver from a donor. Many children do very well long-term after the transplant; ongoing care is required.