Billary Atresia

To diagnose biliary atresia, a doctor will ask about your infant’s medical and family history, perform a physical exam, and order a series of tests. Experts recommend testing for biliary atresia and other health problems in infants who still have jaundice 3 weeks after birth.

If test results suggest that an infant is likely to have biliary atresia, the next step is surgery to confirm the diagnosis.

Doctors may refer children with suspected biliary atresia to specialists, such as pediatric gastroenterologists, pediatric hepatologists, or pediatric surgeons.

Family and Medical history

The doctor will ask about your infant’s family and medical history. The doctor will also ask about symptoms such as jaundice and changes in stool color.

Physical exam

During a physical exam, the doctor may

examine the infant’s body for signs of jaundice

examine the infant’s body for other birth defects that sometimes occur along with biliary atresia

feel the infant’s abdomen to check for an enlarged liver or spleen, which may be signs of biliary atresia

check the color of the infant’s stool and urine

Mother holding an infant while a doctor performs a physical exam

During a physical exam, the doctor may examine the infant’s body for signs of jaundice.

What tests do doctors use to diagnose biliary atresia?

Doctors may order some or all of the following tests to diagnose biliary atresia and rule out other health problems. Doctors may perform several tests because many other diseases can cause signs that are like the signs of biliary atresia.

Blood tests

A health care professional may take a blood sample from the infant and send the sample to a lab. Doctors may use blood tests to measure bilirubin levels and to check for signs of liver disease.

Ultrasound

Ultrasound NIH external link uses a device called a transducer, which bounces safe, painless sound waves off organs to create images of their structure. Using ultrasound, doctors can rule out other health problems and look for signs that suggest an infant may have biliary atresia. However, an ultrasound cannot confirm a diagnosis of biliary atresia.

Hepatobiliary scan

A hepatobiliary scan is an imaging test that uses a small amount of safe radioactive material to create an image of the liver and bile ducts. The test can show if and where bile flow is blocked.

Liver biopsy

During a liver biopsy, a doctor will take pieces of tissue from the liver. A pathologist will examine the tissue under a microscope to look for signs of damage or disease. A liver biopsy can show whether an infant is likely to have biliary atresia. A biopsy can also help rule out or identify other liver problems.

Biliary atresia cannot be treated with medication. A surgery called Kasai procedure (also known as a or hepatoportoenterostomy) creates a path of bile flow from the liver into the intestine.

The surgeon removes the damaged ducts outside of the liver (called extrahepatic ducts). They find smaller ducts that are still open and draining bile. The surgeon then attaches a loop of intestine to this portion of the liver. This allows bile to flow from the remaining healthy bile ducts into the intestine.

After this procedure, infants are usually in the hospital for seven to 10 days to heal. Long-term antibiotic therapy is given to reduce the risk of infection. Additional medications may be used to promote bile flow and help the surgery be successful.

With an experienced hepatologists and surgeons, the Kasai procedure is successful in 60 to 85 percent of the patients. This means that bile drains from the liver and the jaundice level goes down.

The Kasai procedure is not a cure for biliary atresia. It does allow babies to grow and have good health for several, sometimes for many years. About 25% of patients who undergo a Kasai procedure do not need a liver transplant.

Biliary atresia is the most common reason for liver transplantation in children in the U.S. Eighty-five percent of all children who have biliary atresia will need to have a liver transplant before they are 20 years old.

Survival after surgery has increased dramatically in recent years. Children with biliary atresia who have a liver transplant tend to do very well.

Success with the Kasai procedure is related to:

Age. The younger an infant at the time of surgery, the more likely the surgery will be successful. By the time an infant is older than about 3 to 4 months old, surgery is unlikely to be helpful.

Extent of cirrhosis (scarring and damage to liver tissue) at the time of surgery.