Biliary Atresia

If your newborn baby turns yellow and has pale (very light yellow, gray, or white) poop, you might be dealing with biliary atresia. This rare liver disorder shows up within the first few weeks of a baby's life. While it shows up in days for some, for others it can take 2-3 weeks. This liver condition is serious, and it requires both quick initial treatment and long-term follow-up care.

At UVA Health Children’s, our hepatologists (liver experts) have extensive experience helping children with biliary atresia. We can provide the full spectrum of care, from what you need right now to helping your child years down the road.

What Is Biliary Atresia?

The liver makes bile to help break down food, especially fats. Bile normally travels through small tubes called bile ducts from the liver to the gallbladder, where it is stored. When needed, the gallbladder releases bile into the intestines to help with digestion.

When a baby has biliary atresia, bile cannot properly leave the liver. This buildup of bile causes liver damage over time, known as cirrhosis. Symptoms usually begin to appear within the first few weeks of life.

Symptoms of Biliary Atresia

• Yellowing of the skin (jaundice) and whites of the eyes (icterus)
• Pale poop (almost white or gray)
• Dark urine
• Enlargement of the liver (later, especially after a few months of life)
• Big belly; possibly enlarged veins under the skin of the upper belly (later, after a few months of life)
• Poor weight gain (later, after a few months of life)

When we see these symptoms in a baby, biliary atresia is the most common cause. But we still need to rule out other conditions and diseases.

Other conditions that cause liver irritation and/or yellowing of the skin include:

• Infection such as urinary tract infection or congenital infections
• Inherited disorders such as alpha-1 antitrypsin deficiency, Alagille syndrome, cystic fibrosis, and so-called progressive familial intrahepatic cholestasis (PFIC) disorders
• Malformations of the bile ducts
• Tumors of the liver or bile ducts
• Inborn errors of metabolism, including congenital thyroid disease

Diagnosis & Treatment for Biliary Atresia at UVA Health Children’s

UVA Health Children's has the tools to quickly find out if your child has biliary atresia. This is important because treatment is more successful when started early.

Diagnostic Tests

Most of the time, it will take multiple diagnostic tests for a clear diagnosis. Fortunately, we have access to child-friendly imaging and radiologists who work with children every day. That helps limit the number of tests we’ll need to do.

Abdominal ultrasound

Ultrasounds are quick, and give us excellent information about the anatomy of the liver, gallbladder, and bile ducts. And if your child was born without a gallbladder, which is often associated with biliary atresia (absence of the gallbladder can be seen with other disorders as well), we can see that on an ultrasound.

Blood work

Blood work can help us see how well your child’s liver is working and evaluate the cause of the liver disease. That helps us know what our next steps should be.

Liver Biopsy

Ultimately, biliary atresia usually requires a liver biopsy for a conclusive diagnosis. We know this is a scary experience for parents. We use noninvasive tests first to be sure that a biopsy is needed.

Only a tiny sample is needed, and it can be taken with a needle. When we look at this sample under a microscope, it can confirm biliary atresia.

Cholangiography

Cholangiography means taking special pictures of the bile ducts using contrast to fill the bile ducts and x-ray technology. This test needs to be done by a special “interventional” radiologist or a surgeon. Cholangiography shows us whether or not the ducts are open. If they are not, then we will need to proceed with treatment for biliary atresia.

Surgical Treatment for Biliary Atresia

Kasai Procedure

Once we’ve diagnosed biliary atresia, we can schedule surgery. For biliary atresia, a Kasai Portoenterostomy (KPE) procedure will be performed.

The KPE creates a new bile pathway to restore flow. That means the bile your child’s liver is producing can be used for its intended purpose: digestion.  Fortunately the KPE can delay or prevent the need for liver transplantation in many children with biliary atresia. This is especially true if we’re able to perform this procedure in the first 2 months of a baby’s life.

Following this procedure, we’ll keep your child in the hospital to recover. This also gives us time to make sure they’re properly healing and very importantly, that they are getting back to eating well and gaining weight quickly. And we will spend time talking to your family about how we’ll provide ongoing care.

Liver Transplant

Many children born with biliary atresia will need a liver transplant. At UVA Health Children’s, we have one of the top performing pediatric liver transplant programs in the country. We also have a robust living donor program, where people in the community can anonymously donate part of their liver to people in need.