No one knows what causes biliary atresia. About one in 8,000 to 20,000 get this rare disorder.
Trying to figure out what causes this problem or how we can improve life with biliary atresia is one of the highest priorities of the physicians caring for children with biliary atresia at The Children’s Hospital of Philadelphia.
As far as we know, you did not cause your child to get biliary atresia because of anything you did during pregnancy. Nor do you have a gene that gave your child this problem. Within the same family, it is common for only one child in a pair of identical twins to have this disorder.
The surgery for biliary atresia is called a Kasai operation or hepatoportoenterstomy. The purpose of surgery for children with biliary atresia is to restore bile flow.
In the first weeks and months after the surgery, your doctors will closely follow your child to make sure jaundice (yellowness of the eyes and skin) disappears and your child's stools darken.
The most important aspect of care in the first year of life is nutrition. Reduced bile flow and irritation in the liver can sometimes interfere with proper absorption of nutrients and fat provided by standard formulas. To help your child grow, we often use a special type of formula that has easy-to-absorb fat and we also prescribe special vitamins.
Your child may also be prescribed other medicines such as prednisone to decrease the inflammation in the liver, antibiotics to prevent infection of the bile ducts and ursodeoxycholic acid to promote bile flow.
Although the Kasai procedure provides bile drainage for many children, it does not usually cure the child of ongoing inflammation in the liver and bile ducts.
Your child will need close follow-up by a specialist for life. The purpose of these office visits is to watch and, if possible, prevent complications.
Different types of problems develop at different times during your child’s lifetime.
In the first year or two, the major complications are difficulty overcoming the nutritional problems related to the biliary atresia and cholangitis, which is an infection in the bile ducts.
As your child grows, hardening and scarring of the liver might develop. If that happens, there is an increase in the blood flow through certain blood vessels. Hardening of the liver leads to portal hypertension, which is an increased blood flow to vessels that bypass the liver.
In turn, this leads to a number of complications. Some patients develop intestinal or esophageal bleeding. In this situation, blood vessels — especially in the esophagus or stomach — become leaky. When that happens, your child may vomit a large amount of blood. This is always an emergency.
In some cases, a special procedure is performed to stop the bleeding.
Another complication is ascites, which is a collection of fluid in the belly. The treatment is a special medicine to help the patient “pee” out the extra fluid.
Lastly, some children develop an enlarged spleen. When the spleen is large, your physician may prescribe a spleen guard to be worn during gym or during certain activities to protect the spleen from injury.
Approximately half of all children born with biliary atresia will need a liver transplant by the time they are 2 years old. By the age of 20, upwards of 70 percent of patients with biliary atresia will need a liver transplant. Your doctor will consider the possibility of transplantation if there are complications that are not responding to other medical approaches.
Typical reasons to consider a liver transplantation include:
Your doctor will refer you to the liver transplant team before these problems are life threatening. Life after liver transplantation is typically very good.
Yes! Infants with biliary atresia grow up to be children who go to school, participate in school activities and have normal friendships. They will have a scar from their initial surgery, but otherwise look and act normal.
We now care for a group of young adults who were the first generation of patients who underwent a Kasai operation. These adults have jobs and attend college. Worldwide, there are reports of men and women with biliary atresia giving birth to healthy children.
There are a number of organizations and groups that can provide more information about biliary atresia. For details, see our resources for families.