What is biliary atresia?
Biliary atresia is a liver condition. It occurs when a baby's bile ducts do not form normally. Instead of being open tubes to drain bile from the liver, the bile ducts are missing or they are blocked because of scarring.
Bile is a liquid made by the baby's liver. It helps the body digest (break down) fats. Normally, bile flows through tiny bile ducts within the liver into larger ducts that lead to the gallbladder and intestine. The gallbladder stores bile until the body needs it to digest food. Food triggers the gallbladder to contract and squeeze bile out into the intestine.
In babies with biliary atresia, the abnormal or scarred bile ducts keep bile from flowing out of the liver. Instead, bile backs up in the liver. Backed-up bile (congestion) in the liver is called cholestasis. It causes scarring (cirrhosis) and jaundice, or a yellowish tint to the skin and eyes. This is because bile contains a yellow pigment, called bilirubin.
Biliary Atresia in Children
About 1 baby in 15,000 has biliary atresia. Doctors do not know why it occurs. In most cases, it is present early in infancy. Usually it's found in the first one to two months of life. If it's not found until after 2 months of age, it seems to progress to worse disease.
The bile ducts may be abnormal when the baby is born (congenital). In some babies, the bile ducts may not form correctly while the baby is developing. A small number of babies with biliary atresia (about 15%) may have other structures that did not form normally - their intestine, spleen and portal vein, which goes to the liver.
In some babies, the ducts form but cells that cause inflammation (swelling) may collect around the ducts. The inflammation may make the ducts swollen and narrower, closing them off and creating scars.
In order to survive, babies with biliary atresia need surgery. This is usually done in the first two months after birth. The goal is to remove any blockage in the ducts outside the liver and create a way for bile to drain straight from their liver into their intestines. This surgery is called the Kasai procedure. It is named after Dr. Kasai of Sendai, Japan, who developed the surgery in the 1960s.
Without surgery, biliary atresia leads to liver failure. A baby with liver failure may be able to have a liver transplant. Biliary atresia is the most common reason for liver transplants in children. It accounts for nearly half of them.
Biliary Atresia at Seattle Children's
We have treated many babies with biliary atresia. Our surgeons are experienced at performing the Kasai procedure that creates direct drainage for bile from the liver into the intestine (portoenterostomy). Each year, our surgeons perform four to six of these surgeries.
When you come to Seattle Children's, you have a team of people to care for your child before, during and after surgery. Along with your child's surgeon, you are connected with doctors trained to care for newborns with complex problems (neonatologists), doctors who specialize in gastrointestinal (stomach and intestine) and liver diseases, nurses, dietitians, child life specialists, social workers and others. We work together to meet all of your child's health needs and help your family through this experience.
Since 1907, Seattle Children's has been treating children only. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board-certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age. Our expertise in pediatrics truly makes a difference for our patients and families.
At Seattle Children's, we also have the only pediatric liver transplant program in the Pacific Northwest. The outcomes of our well-regarded program are among the best in the nation.