Guide to Biliary Atresia

Biliary atresia is a rare condition that prevents the liver from draining bile to the intestines. This causes bile to build up in the liver, which can produce scarring and eventually liver failure.

Key Facts

Bile is a fluid produced by the liver that helps with digestion. Normally, bile secretes to the intestines through bile ducts. Biliary atresia prevents this by blocking the bile ducts.
Biliary atresia only affects infants. It usually appears about two to eight weeks after birth.
Without treatment, biliary atresia can be fatal. While there are no medications available for this condition, there are several surgical treatments.

Causes

The causes of biliary atresia are still not well understood. Researchers currently believe it may be caused by several factors, such as undeveloped bile ducts, a viral or bacterial infection, an autoimmune response from the body, or environmental factors, such as exposure to toxins.

The following are known to increase the risk of developing this condition:

Premature Birth: Infants born before 37 weeks are more likely to develop biliary atresia.
Race: African-Americans and Asians have higher rates of this condition.
Gender: Women are slightly more likely to develop this condition than men.

Biliary atresia is not an inherited condition, nor has it been linked to any medications mothers may take during their pregnancy. It remains a rare condition, occurring in only about one in every 15,000 live births.

Symptoms

Biliary atresia usually does not develop until two to eight weeks after birth. When it does, the following are some of its most common symptoms:

Clay-colored stools
Dark urine
Jaundice (yellowing of skin and eyes)
Swollen abdomen
Weight loss

Diagnosis

Because biliary atresia can resemble several other conditions, doctors use several methods to diagnose it. They usually begin by evaluating liver function through blood tests. These measure the following:

Bilirubin: Bilirubin is a waste product that is processed by the liver. If bilirubin levels are high, it may be a sign that something is wrong with the liver.
Blood Clotting: If blood takes longer than usual to clot, it may be a sign of blocked bile ducts.
Liver Enzymes: High levels of certain enzymes, such as alkaline phosphatase, may indicate liver damage.
Proteins: Albumin and globulin are two proteins made by the liver. Low levels may mean the liver is not functioning correctly.
Viruses: The presence of hepatitis or HIV could indicate biliary atresia.

The following are some other common ways to diagnose biliary atresia:

Diagnostic Surgery: The surgeon will make an incision in the child’s abdomen in order to look at the liver and bile ducts and make a diagnosis.
Imaging Tests: These will allow doctors to form an image of the liver so they can better detect signs of biliary atresia. They may include an x-ray or an ultrasound. Doctors may also do a hepatobiliary scan, which involves injecting a radioactive isotope into the veins and using a nuclear scanning machine to see if it passes through the bile ducts or not.
Liver Biopsy: This is a surgical procedure that removes a tiny portion of liver tissue so that doctors can examine it under a microscope to determine what is wrong.

Treatment

Biliary atresia cannot be treated with medications. Instead, the following two surgical operations are the only viable treatments:

Kasai Procedure

This surgery bypasses any blocked bile ducts by connecting the liver directly to the intestinal tract. First, the surgeon identifies and removes any blocked bile ducts located outside of the liver. Next, they identify bile ducts within the liver that are still draining bile. They then take the small intestine and connect it directly to those bile ducts, allowing bile to drain from the liver.

This procedure is not considered a cure for biliary atresia. Instead, it can improve the flow of bile for several months or even years, after which a liver transplantation may be necessary. Success rates for this procedure are about 80 percent for infants eight weeks and younger. Children older than four months are unlikely to benefit from this surgery.

Liver Transplantation

This procedure involves removing the entire diseased liver and replacing it with a healthy donor liver. The donor liver can either come from a deceased donor or from a small portion of a living donor’s liver. After several months, this small portion will then regenerate into a fully functioning liver.

Learn more about liver transplantations and living donor liver transplantations.

Outlook

Although biliary atresia is a serious and potentially fatal condition, surgical advances have made the long-term outlook for this disease good. For instance, the Kasai procedure can reestablish full bile flow in about 25 percent of children and partial bile flow in about 50 percent of children.

While some of these children will be able to retain their native liver, the majority will eventually need a liver transplantation. According to data from the Organ Procurement and Transplantation Network (OPTN), the national survival rate for deceased donor liver transplants between 2008 and 2015 was:

91 percent one year after surgery
83 percent three years after surgery
75 percent five years after surgery

Note: These statistics are the average survival rate for all ages, including the very old and very young, as well as the critically ill. It is not unusual for recipients to lead normal lives for 30 years or more after an operation.

After transplantation surgery, most patients will need to take anti-rejection and anti-infective medications for the rest of their lives.

Next Steps

Our program is staffed by specialists with deep experience identifying, diagnosing, and treating biliary atresia in young children. We are also a nationally recognized leader in both liver transplantation and living donor liver transplantation.

If your child has been diagnosed with biliary atresia and you'd like to learn more about the latest treatment options, we can help. We accept a number of insurance plans, and our team can help confirm your coverage. Contact the Columbia Center of Excellence for Biliary Atresia by calling (212) 305-3000 or completing our online appointment form.