By: David T. Schindel, MD FAAP; Humberto Lugo-Vicente, MD
Biliary atresia is a
rare liver condition that affects babies. Because of inflammation and scarring in the bile ducts, digestive fluids (bile) can't drain from the liver into the intestines. Most infants with biliary atresia are full-term and otherwise healthy. The baby will often have
jaundice, pale stools and dark urine by 4 to 6 weeks of age.
What problems does biliary atresia cause?
Because bile is blocked from draining from the liver into the intestine with biliary atresia, a pigment called bilirubin, and other substances found in bile, collect in the liver, blood and other organs, including the brain. As these substances build up, the liver and brain can be injured.
What are some signs or symptoms of biliary atresia?
Failing to gain weight and developing jaundice are early signs that a baby may have biliary atresia. Jaundice is what you see when bilirubin collects in the tissues. People with jaundice have gradual yellowing discoloration of the skin and white parts of the eyes. Another common finding is that the child's stools, which are normally yellow, green, or brown because they contain bilirubin, become pale. Often, along with abnormally pale stools, the urine becomes very dark or amber in color.
What tests will be done to diagnose biliary atresia?
If you have any concerns, your baby should be seen by their pediatrician as soon as possible. Following an examination, blood will be drawn to check liver function as well as other vital functions. An ultrasound of the abdomen is recommended to look for the bile ducts and gallbladder. If these structures are not well-visualized, further advanced studies, such as a hepatobiliary iminodiacetic acid (HIDA) scan, may be needed to confirm bile ducts are present and normal.
What can be done to make my child better?
If test results suggest a diagnosis of biliary atresia, based on increased bilirubin and missing gallbladder or bile ducts on a HIDA scan, surgery is recommended.
In the operating room, a cholangiogram is performed to confirm the diagnosis. This is done either through a small incision in the child's upper abdomen or through a laparoscope. To perform a cholangiogram, the surgeon places a small tube into the gallbladder, if present, and injects a medication that can be used to see the bile ducts using an X-ray machine.
If bile ducts are not seen connecting the liver to the intestine, the diagnosis of biliary atresia is confirmed. At this point, the surgeon will perform a procedure that re-establishes a connection between the liver and the intestine to allow drainage of the bile from the liver into the intestine. This procedure is called a Kasai operation.
Remember
Regular visits with your child's pediatrician can help diagnose biliary atresia early and prevent related complications.
More Information
About Dr. Schindel
 David T. Schindel, MD, FAAP, a pediatric surgeon with Pediatric Surgical Associates, works out of Medical City Children’s Hospital in Dallas. Dr. Schindel's clinical and research interests include congenital surgery of the newborn, such as biliary atresia, choledochal cysts, abdominal wall defects, lung malformations and tracheoesophageal surgery. He also has significant interest and experience in the surgical management of pediatric Inflammatory Bowel Disease. He is a fellow of the American Academy of Pediatrics and the American Pediatric Surgery Association.
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About Dr. Lugo-Vicente
 Humberto Lugo-Vicente is Professor of Pediatric Surgery at the University of Puerto Rico, Universidad Central del Caribe, and Ponce Schools of Medicine. He is the Director of Pediatric Surgery at San Jorge Children's Hospital and the chief editor of
Pediatric Surgery Update (http://pedsurgeryupdate.com/).
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