Biliary Atresia in Babies – Causes, Symptoms & Treatment

Biliary atresia is a rare liver disease that affects newborn babies. It can hamper the proper functioning of the liver. This condition can sometimes lead to fatal or life-threatening complications in babies. Biliary atresia affects only infants and some babies get it in the womb. Its symptoms appear when a baby is between 2 and 4 weeks after birth. Read this article to know more about this liver ailment. Find out how it occurs, its symptoms, and how it can be treated.

What Is Biliary Atresia?

Biliary atresia or extrahepatic biliary atresia is a liver disorder that occurs in infants or newborn babies soon after birth and in some cases even before birth. This disease affects the bile ducts or the tubes inside the liver and if left untreated, it can lead to some serious complications and fatalities.

The cells in the liver produce a fluid known as bile, which helps in digestion of fat and carries waste from the liver to the intestine for excretion. In the normal course, the biliary system, which consists of the network of these ducts and channels, helps in draining out the bile from the liver to the intestine. However, when a baby suffers from biliary atresia, these bile ducts get blocked in the liver. This leads to the accumulation of bile in the liver that can lead to scarring and damaging liver cells. This trapped bile can affect various other vital functions of the body and sometimes even result in liver failure.

How Common Is It?

As mentioned it is a rare disorder. It can affect one in 15,000 babies. However, this is the most common liver disorder in babies that may require a liver transplant.

Which Babies Are At a Greater Risk?

The actual reason why this condition occurs is not known, however, there are certain factors that may put some babies at a greater risk than others. This rare liver ailment that can affect one in every 15000 to 20000 babies and is more likely to occur in preterm or premature babies. This disorder is also seen as more prominently in female babies than male ones. Also, poor maternal nutrition, substance abuse, or congenital malformations can put a child at greater risk.

Types of Biliary Atresia

Biliary atresia can be broadly classified into two categories depending on when these conditions develop:

1. Perinatal Biliary Atresia

This type is the most common type of biliary atresia that usually occurs in the majority of the cases. This condition occurs within two to four weeks after birth.

2. Fetal Biliary Atresia

This is not a very common type and can occur only in very few cases. This is the kind of biliary atresia that occurs while the baby still grows inside the mother’s womb.

Causes of Biliary Atresia

The exact cause of biliary atresia is not known. Here are some reasons that can cause this complication in babies:

• Some kind of congenital deformities can cause biliary atresia in babies, though such occurrences are very rare.
• Sometimes genetic disorders or infections that result in immune dysregulation may also cause this condition in babies.
• Exposure to harmful or toxic chemicals after birth may lead to this ailment disease.
• Some kind of genetic mutation that leads to permanent changes in gene structure may result in this condition.
• Bacterial or viral infections can also cause biliary atresia in infants.

The gene mutation may result in autoimmune damage, where the immune system may result in affecting or destroying the bile duct. However, it is important to understand that this disease cannot be inherited by babies, which means parents cannot pass it on to their babies.

Biliary Atresia Symptoms

The symptoms or signs of biliary atresia in infants usually surface around two to four weeks after birth. This means that at birth the baby may look normal and healthy. Here are some symptoms that you should keep an eye on:

• The baby may develop jaundice, which can make the baby’s skin and eyes appear yellow in colour. This happens because of the presence of high levels of bilirubin in the blood. Though newborn babies may usually develop jaundice because of the immature liver, but it subsides within a week to ten days time. But if the baby has biliary atresia, he may develop jaundice two to three weeks after birth.
• The baby may pass clay-coloured, also known as acholic stools. This happens because of the absence of bilirubin in the intestines.
• The enlarged and firm liver may cause swelling in the abdominal region of the baby.
• The baby may pass dark coloured urine. This occurs due to the bilirubin buildup in the blood.
• The baby may not gain height or weight because the baby may not feed properly. This may also lead to irritability and fussiness in babies.

How Is Biliary Atresia Diagnosed?

In case your baby shows jaundice like signs and symptoms for more than two to three weeks, your doctor may make use of some of the following procedures to establish biliary atresia in babies:

• The doctor may conduct a physical examination to examine the yellowing of the skin and eyes of the baby. The doctor may also check the baby’s tummy to examine any signs of swelling.
• The doctor may conduct stool and urine tests to see the traces of excessive secretion of bilirubin.
• Blood tests will be conducted to check the bile levels. High amounts of bilirubin are an indication of improper liver functions.
• The doctor can conduct a biliary atresia ultrasound scan to establish any anomalies in the liver.
• An x-ray can be conducted to check for an enlarged spleen and liver.
• A biliary atresia Hida scan, also known as the hepatobiliary scan can be performed, which involves inserting very small amounts of a radioactive substance, to obtain a clearer x-ray image of the bile ducts and liver.

Biliary Atresia Treatment

Biliary atresia cannot be cured with medication; however, surgical procedures can help in managing the condition. The surgery cannot only reduce the intensity of the disease but it can also slow its progression. Here are the surgical procedures that can be suggested by the doctor:

1. The Kasai Procedure

The Kasai hepatoportoenterostomy or Kasai procedure for biliary atresia involves re-establishing the bile flow. The procedure involves removing the entire bile duct and replacing it with a part of the small intestine that is rolled up in a loop and attached to the liver to assist the flow of bile into the small intestine. There can be different variations to this procedure depending on the severity of the condition or damage to the liver. This procedure has a high success rate if conducted within the first three months after birth. There are about fifty per cent chances of developing infection post the surgery, which can be controlled by administering antibiotics. However, if the infection gets out of control or the procedure is unsuccessful, in that case, the doctor may recommend going in for the liver transplant.

2. The Liver Transplant Procedure

In case the blockage is present in the bile ducts inside the liver or the Kasai procedure is unsuccessful, the doctor may recommend the liver transplant procedure. Sometimes a baby may require a transplant even after a successful Kasai procedure. This can occur when there are persistent liver-related problems or issues after the Kasai procedure. The liver transplant can be partial or complete, depending on the severity of the damage to the organ.

What Complications Can Occur If Surgery Is Not Done?

If left untreated or if surgery is not conducted for biliary atresia, it can lead to fatal and life-threatening conditions in babies. High amounts of bilirubin in the body can lead to severe itching, which can be very uncomfortable and distressing for the young babies, however, medicines may provide some relief in managing this condition.

Another condition that may occur is portal hypertension, which surfaces because of the damage caused to the liver. Bacterial cholangitis is a complication that can affect babies, which causes discoloured stools and fever in babies. Hampered liver functioning also leads to malabsorption of vitamins and fats, which may lead to stunted growth in babies.

When to Call a Doctor

Following are some of the reasons that call for concern and you should report the same to your doctor:

• Your baby passes dark-coloured or brown-coloured urine.
• Your baby passes pale or whitest coloured stools.
• Your baby’s skin and eyes appear yellow in colour.
• Your baby’s tummy appears swollen.
• Your baby is not gaining weight and height.

How Can Parents Help Their Child Live With Biliary Atresia?

Your baby will need special care before the treatment procedure begins. One of the main concerns is helping the baby to get nutrition because damage to the liver can interfere with protein and vitamin absorption by the body. Kids with this liver disease need more amounts of calories because of their faster metabolism, in comparison to other same-age children.

Your doctor may recommend meeting a paediatric nutritionist, who can help you guide your child’s nutritional needs and what you do to compensate for the same. A diet plan can be chalked out that may include all the required vitamins, minerals, and other nutrients in the right amounts.

In case, your child cannot eat normally or is too sick to eat food the normal way, the doctor may suggest giving calorie-dense fluids that can be fed through an NG tube or nasogastric tube. This tube is inserted into the child’s nose and through the oesophagus to reach the stomach. This is a great option for kids who are too sick to eat or can only eat very small amounts of food at a time.

Even after the surgery is performed, your child will require special care with the diet until the child’s digestion gets back to normal. Your healthcare practitioner may recommend added vitamins or supplements. How fast your child recovers from the surgery may depend on many factors, which may usually include your kid’s overall health, the amount of damage to the bile ducts, the age of the child, the extent of liver damage, etc. However, with the passage of time, your child’s condition will improve but you must make sure that your child follows the medical routine strictly. Therefore, work closely with your doctor on keeping a tab on your child’s health for a speedy recovery.

Can Biliary Atresia Be Prevented?

This liver disorder is something that cannot be prevented because the exact cause cannot be established. However, there are some measures that can be taken to reduce the risk factors of this fatal condition. The mother should take optimum care of her nutritional needs and maintain a healthy lifestyle (no drinking, no smoking, and any other kind of substance abuse) to ensure optimal fetal growth and development. Also, regular and comprehensive screenings during pregnancy for any kind of infection and diseases can keep this condition at bay.

Maintaining proper hygiene and exclusively breastfeeding the baby can help in reducing the risk too. Getting your baby immunized against infections such as rotavirus can prove to be helpful too. However, if the ducts are already blocked, there is nothing much that you can do.

Biliary atresia can adversely impact your baby’s health. However, the latest advancements in the medical field can help in ensuring better chances of having a normal and healthy life for your little one. Therefore, as soon as you register any symptoms of biliary atresia is your baby; get in touch with your doctor. With the right treatment, lifestyle changes, and care, you can manage this serious health condition in your baby.

Talk to your doctor to know more about this health condition in babies and what can be done to manage this disorder.

Also Read:

Tay-Sachs Genetic Disorder in Infants
Most Common Health Issues and Diseases in Babies
Neonatal Hepatitis