Biliary Atresia

Some babies may develop intense itching, or "pruritus," which makes them extremely uncomfortable and irritable. The exact cause of this itching is not yet known, although researchers have found a connection between it and the backup of bile. The cause of biliary atresia has not yet been discovered.

The disease affects approximately one infant in every 20,000 live births. Girls are affected slightly more often than boys, but no racial or ethnic group appears to be more affected than any other.

Biliary atresia is not a hereditary condition (although in some very rare cases, more than one infant in a family may be affected). Many parents experience feelings of guilt, but they should be reassured that nothing they have done caused their child's illness. There are many liver diseases which cause symptoms similar to those of biliary atresia. Consequently, many tests may have to be performed before biliary atresia can be diagnosed conclusively.

Every effort should be made to search for any of the causes of jaundice which might be confused with biliary atresia. This involves blood and urine tests; liver function tests; blood counts and a test for clotting function. A painless examination using ultrasound (ECHO) is often done to study the liver and determine the size of the bile ducts and gallbladder.

Other tests which are often used are specialized X-ray techniques or radioactive scans of the liver which can be helpful in focusing on the true abnormality. A liver biopsy, in which a tiny sample of the liver is removed with a needle, allows the physician to examine the liver tissue microscopically. The most successful treatment for biliary atresia to date is a type of surgery which creates drainage of bile from the liver when the ducts have become completely obstructed. This operation is called the Kasai procedure (hepatoportoenterostomy) after Dr. Morio Kasai, the Japanese surgeon who developed it.

In the Kasai procedure, the surgeon removes the damaged ducts outside of the liver (extrahepatic) and replaces them with a length of the baby's own intestine, which acts as a new duct.

The aim of the Kasai procedure is to allow excretion of bile from the liver into the intestine via the new duct. The operation accomplishes this about 50 percent of the time. In those who respond well, jaundice usually disappears after several weeks.

In the remaining 50 percent of cases where the Kasai procedure does not work, the problem often lies in the fact that obstructed bile ducts are "intrahepatic" or inside the liver, as well as outside. No procedure has yet been developed to correct this problem except for transplantation. The aim of treatment after surgery is to encourage normal growth and development. If bile flow is good, the child is given a regular diet. If bile flow is reduced, a low fat diet is recommended as bile is required to aid in the absorption of fats and vitamins. Multiple vitamins, vitamin B complex, and vitamins E, D, and K can be given as supplements. Unfortunately, despite bile flow, the Kasai procedure is not a cure for biliary atresia. For reasons which are still unknown, liver damage often continues and, eventually, cirrhosis and its complications appear. Patients with cirrhosis have changes in blood flow through the liver which may produce abnormalities, such as easy bruising of the skin, nosebleeds, retention of body fluid, and enlarged veins, called varices, in the stomach and esophagus. Increases in pressure in these veins can make them leaky and internal bleeding results. This can usually be stopped. In some cases, a procedure may be required whereby a hardening (sclerosing) agent is injected into these veins.

As the disease progresses, other complications may occur. While all infants tend to be sleepy after eating, those with biliary atresia may experience excessive sleepiness after eating protein, due to increased nitrogen products in the bloodstream. The child may also suffer from an increased risk of infection. Following the Kasai operation, infection in the bile ducts (cholangitis) is common. This is usually treated initially using intravenous antibiotics and may be continued with oral antibiotics.

If retention of body fluid occurs, it can be treated with diuretics and potassium replacement.

Jaundice or itching can often be treated successfully with medications (phenobarbital, cholestyramine and ursodeoxycholic acid). The extent and type of liver damage differ in each baby with biliary atresia. Some infants respond to the Kasai procedure; others do not. If bile continues to flow, long-term survival is possible. However, it is presently impossible for a physician to determine in advance which baby is likely to respond to treatment. Liver transplantation is an option which is becoming increasingly useful to victims of certain liver diseases. The survival rates for transplant recipients have increased dramatically with improved surgical techniques and the development of new drugs which help overcome the problem of organ rejection.

In children with biliary atresia, liver transplantation is generally not attempted until the Kasai procedure has been performed. If this operation is not successful, and before complications of the resulting cirrhosis become severe and life threatening, liver transplantation may be attempted. It has been successful in numerous cases. However, as in all organ transplantation, success depends greatly upon the timely availability of suitably matched organs for donation, the time factor involved (a donated liver must be used within 16 hours for the operation to be successful), and other factors which are only now being investigated. The use of reduced-size and living-related transplants are aiding in the timing and availability of suitable donor organs. Watching a young infant suffer from biliary atresia is a devastating experience. It can also be frustrating, because so little is known about the disease. Feelings of anger and helplessness are not uncommon. What does one do when there is so little one can do?

Many parents have found it helpful to learn as much as they can about the disease. Talk to your physician, inquire about specialists and request any literature on the subject.

Perhaps the biggest comfort for parents is to discuss the problem with others who have or are going through a similar tragedy. Finding out that they are not alone, that others feel the way they do, and learning how other parents are coping with their child's disease, is often a great comfort. The American Liver Foundation recognizes that parents of children with biliary atresia need help in coping with the immense strain of this chronic illness. To meet this need, the American Liver Foundation is continually organizing and coordinating mutual help groups through its chapters to provide emotional support for families, making referrals to specialists where appropriate, and keeping people aware of the latest research developments. There can be no cure for biliary atresia until the cause of the disease can be determined. Researchers are focusing on trying to find this cause, but a great deal of work still needs to be done. More research into how the liver works is also vital.

Research is the key that will unlock this mystery. The American Liver Foundation is the only national voluntary health agency dedicated to funding research and helping people understand more about the liver and liver disease.