Biliary Atresia in Children: Diagnosis and Treatment

How is Biliary Atresia Diagnosed?

It is important to establish an early diagnosis of biliary atresia. This increases your baby’s chances of successful treatment. If your baby has jaundice for more than 4 weeks after birth, it is important that they are tested for biliary atresia or other liver problems, including congenital infections and genetic disorders that may also result in increased levels of bilirubin in the blood.  

Your baby’s doctor may do some of the following tests to check for biliary atresia:

• Blood tests to check liver function (how well the liver is working). These tests look for high levels of liver enzymes (proteins that speed up chemical reactions in the liver) or bilirubin (a substance that appears during the normal break down of red blood cells). High levels of these substances mean that the liver is not working well.
• Tests that measure how long it takes for the blood to clot. The liver is an important part of normal blood clotting. If the liver is very damaged, blood clotting will be affected.
• HIDA/hepatobiliary scan. This test uses a low dose of radiation injected in the baby’s veins to track the formation of bile in the liver and the subsequent delivery of bile into the intestine.
• Liver biopsy. This is when a small piece of the liver is removed and checked under a microscope.
• X-Rays and ultrasounds of the abdomen. These imaging tests allow doctors to check for abnormalities (unusual changes) in the liver and gallbladder.
• Operative cholangiogram. This would happen during diagnostic surgery, where doctors check for damage to the liver or bile ducts. It involves injecting dye or a low dose of technetium (a type of radiation) into the liver and seeing if it flows normally out of the bile ducts.

How is Biliary Atresia Treated?

Doctors can treat biliary atresia in the following ways:

High-nutrient, high-calorie diet

It is common for children with biliary atresia to have trouble absorbing nutrients and getting enough calories from food. The lack of bile flow from the liver to the intestines makes it harder for your child to absorb fats. Before your child has surgery to treat biliary atresia, nutrition might be an issue for them. Liver damage can also affect your child’s protein levels and metabolism (process that helps the body use food for energy).

It is important for your child to eat a diet that is higher than usual in nutrients and calories. If your child has trouble eating, they may need high-calorie liquids instead of traditional meals to receive necessary nutrients.

Kasai Procedure

A Kasai procedure creates a path for the bile to flow from the liver into the intestine. The surgeons also remove the gallbladder and diseased bile ducts to connect the new, healthy opening on the undersurface of the liver to the small intestine. This surgery often slows the pace of the disease. However, in the majority of cases, the Kasai procedure is not a long-term cure. Over time, bile continues to accumulate inside the liver, injuring the surrounding liver cells (hepatocytes). Many children with biliary atresia will eventually need a liver transplant, even if they have undergone the Kasai procedure. Even if they do not need a liver transplant, many patients will experience ongoing liver injury during their lifetime.

A Kasai procedure is most helpful if performed during your baby’s first 3 months of life. When successful, the surgery can help your child be healthy for several years..

Possible complications following the Kasai procedure include:

• Cholangitis (infection/inflammation of bile ducts in the liver)
• End-stage cirrhosis (causing a need for a liver transplant)
• Chronic liver disease
• Intestinal obstruction

Liver Transplant

If a Kasai procedure is not successful, your child may need a liver transplant. By the time that children who have undergone the Kasai procedure turn 20, a liver transplant will be needed in 8 out of every 10 cases. A liver transplant is the only cure for liver injury associated with biliary atresia.

During a liver transplant, the surgeon removes the damaged liver and replaces it with liver from one of the following sources:

• a whole liver from a deceased donor
• a part of a liver from a deceased donor
• part of a liver from a living donor whose tissue type matches your child’s tissue type. A living donor is usually a relative.

Possible complications following liver transplant include:

• Failure or rejection of donated liver
• Bile duct complications
• Infection
• Bleeding
• Blood clots

Outlook

Long-term survival for biliary atresia patients is excellent. Currently, 85-90% of children with biliary atresia are expected to survive into adulthood. Though some babies die from associated embryonic or fetal problems, the Kasai procedure and pediatric liver transplants have made biliary atresia a more manageable condition. Biliary atresia patients can be cured by liver transplants and lead healthy adult lives. Those who do not need liver transplants by age twenty may face liver problems later on that can be managed with medicine.