Biliary atresia cannot be treated with medication. A Kasai procedure or hepatoportoenterostomy is done. The Kasai procedure is an operation to re-establish bile flow from the liver into the intestine. It is named after the surgeon who developed it.
The surgeon removes the damaged ducts outside of the liver (extrahepatic ducts) and identifies smaller ducts that are still open and draining bile. The surgeon then attaches a loop of intestine to this portion of the liver, so that bile can flow directly from the remaining healthy bile ducts into the intestine.
After this procedure, infants are usually in the hospital for seven to 10 days to heal. Long-term antibiotic therapy is given to reduce the risk of infection, and additional medications may be used to promote bile flow and maximize the success of the operation
With an experienced surgeon, the Kasai procedure is successful in 60 to 85 percent of the patients. This means that bile drains from the liver and the jaundice level goes down.
The Kasai procedure is not a cure for biliary atresia, but it does allow babies to grow and have fairly good health for several, sometimes for many, years.
In 15-40 percent of patients the Kasai procedure does not work. If this is the case, liver transplantation can correct this problem.
Success with the Kasai procedure is related to:
- Age. The younger an infant at the time of surgery, the more likely the surgery will be successful. By the time an infant is 4 months old, surgery is unlikely to be helpful.
- Extent of liver damage (cirrhosis) at the time of surgery.
- The number and size of microscopic ducts in the scarred tissue that can drain bile.
- The experience of the surgical and medical team. Centers with teams made up of specialists with extensive experience have success rates that are greater than those centers with less experienced teams.
Nutrition and Biliary Atresia
Children with liver disease have a faster metabolism than healthy children. This means that children with biliary atresia may require more calories.
A child with biliary atresia and jaundice cannot properly digest fats. This is because not enough bile gets to the intestine. Due to liver damage, there may also be a loss of vitamins and protein.
Guidelines from your doctor for your child's nutrition may include:
- A well-balanced diet, consisting of three meals a day plus small snacks in between meals
- Vitamin supplements
- Adding medium-chain triglyceride (MCT) oil to foods and liquids or infant formulas. MCT adds extra calories that will help your child grow.
- High-calorie liquid feedings may be recommended if your child is too ill to eat normally. Feedings are given through a special tube (nasogastric tube) that is placed in the nose and guided down the esophagus and into the stomach.
Although digestion may return to normal after surgery, extra vitamins or MCT oil may be needed.
What Are the Complications of Biliary Atresia and What Can Be Done for Them?
Complications right after surgery are low. Most problems that develop are because the biliary atresia is getting worse.
- After the Kasai procedure, it is common to get an infection in the bile ducts. This is usually treated using intravenous antibiotics. Treatment may continue with oral antibiotics.
- Jaundice or itching may occur. These can often be treated successfully with medications such as cholestyramine and ursodeoxycholic acid (for itching).
- Many patients with cirrhosis have changes in blood flow through the liver and intestines. These changes may produce problems such as easy bruising of the skin, nosebleeds, retention of body fluid and enlarged veins (varices) in the stomach and esophagus.
Increased pressure in these veins can cause a sudden and large amount of bleeding in the stomach and intestines. Although this can be a very serious complication, with prompt and experienced medical care, bleeding can usually be stopped. Sometimes that requires specialized procedures in which a hardening (sclerosing) agent is injected into the abnormal vessels.
- If retention of body fluid occurs, it can be treated with diuretics (“water pills”).
As the disease gets worse, other complications of cirrhosis may also occur.