Biliary atresia cannot be treated with medication. A Kasai procedure or hepatoportoenterostomy is done. The Kasai procedure is an operation to create an open duct so bile can drain from the liver. It is named after the surgeon who developed it.
The surgeon removes the damaged ducts outside of the liver (extrahepatic ducts) and replaces them with a piece of the baby's own intestine. This new duct allows bile to pass from the liver into the intestine.
After this procedure, infants are usually in the hospital for seven to 10 days to heal. Medications are used to prevent excessive fluid buildup in the abdomen (ascites). Long-term antibiotic therapy is also given to reduce the risk of infection.
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 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 years.
When this procedure does not work, it is usually because the blocked bile ducts are inside the liver (intrahepatic), as well as outside the liver (extrahepatic). If this is the case, liver transplantation can correct this problem.
Success with this procedure is related to:
- Age. Surgery is most successful in infants younger than 2 to 3 months of age.
- 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 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 phenobarbital (for jaundice), 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 large amount of bleeding in the stomach and intestines. Though this can usually be stopped, in some cases a hardening (sclerosing) agent is injected into these veins.
- If retention of body fluid occurs, it can be treated with diuretics and potassium replacement.
- Due to increased protein products in the bloodstream, infants with biliary atresia and cirrhosis may be very sleepy after eating protein.
As the disease gets worse, other complications of cirrhosis may also occur.