Biliary atresia is an uncommon disorder in which a normal baby develops progressive destruction of the gallbladder and biliary system after birth.  A less common form of biliary atresia occurs as part of a syndrome of abnormalities prior to birth. The cause of biliary atresia is not known.  Biliary atresia causes progressive liver dysfunction. Symptoms include: jaundice, easy bleeding and bruising, pale stools, dark urine, edema, and poor growth.  Infants with early diagnosis of biliary atresia may benefit from a surgery called a Kasai procedure.  This procedure involves connecting the intestines to the liver to allow normal flow of bile.  Most infants with biliary atresia will require a liver transplant.  Surprisingly, most children with liver transplants do quite well.

What is biliary atresia and what causes it?

Biliary atresia is a disorder in which the gallbladder and bile ducts are gradually destroyed.  There are two basic types of biliary atresia: the prenatal type and the postnatal type. The prenatal type of biliary atresia is typically associated with a congenital syndrome with multiple other abnormalities. The more common postnatal type develops after birth in an otherwise normal infant.  The cause of biliary atresia is not known.

What are the symptoms of biliary atresia? 

• Jaundice 
• Easy bleeding and bruising
• Pale stools 
• Dark yellow urine 
• Edema
• Poor growth 

How is biliary atresia diagnosed?

• More common causes of jaundice must be ruled out first. 
• Physical exam will likely reveal an enlarged liver and spleen 
• Edema may be present including ascites which is fluid swelling of the belly 
• Imaging studies including ultrasound and CT scan will reveal an enlarged liver and spleen including pouring visualization of the gallbladder and bile ducts.

Blood tests

• An elevated total bilirubin level
• A high proportion of the direct or conjugated form of bilirubin
• Elevated liver enzymes 
• Elevated INR (a test that measures blood clotting function) 
• Multiple others 

Imaging studies

Abdominal ultrasound will show a large liver and spleen and pour visualization of the gallbladder in bile ducts. 

A HIDA scan will reveal poor flow of bile into the intestines. 

Surgical procedures

Liver biopsy will reveal excess bile ducts and liver inflammation.

An exploratory laparotomy and  intraoperative cholangiogram will reveal poorly formed or damaged bile ducts. 

How is biliary atresia treated? 

It biliary atresia is diagnosed early in life (before about four months of age), the infant may benefit from a procedure called a kasai procedure in which the intestines are attached to the surface of the liver to replace the bile ducts. 

Most infants with biliary atresia will ultimately require liver transplantation. 

How successful is liver transplantation in children? 

Approximately 90% of children with liver transplantation will survive more than five years.  Many children are surviving for much longer.    These children will require medications daily for lifetime but can grow to have relatively normal lifestyles. 

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