Treatment for Your Child's Biliary Atresia: Kasai Procedure

Treatment for Your Child's Biliary Atresia: Kasai Procedure

Biliary atresia is a serious liver problem that occurs in young infants. It involves a problem with the bile ducts (the tubes through which bile drains from the liver into the small intestine). In children with biliary atresia, bile ducts are damaged, missing, or not shaped correctly. Treatment must be done as soon as possible. Biliary atresia is treated with surgery. Even if this surgery goes well, the child will likely need a liver transplant sometime in the future.

The Kasai Procedure

Surgery for biliary atresia is called the Kasai procedure. During this surgery, the surgeon removes any problem bile ducts outside the liver. The small intestine is then attached to the liver. This provides a path that can allow bile to drain from the liver. The exact details of the procedure for your child will depend on the condition of your child's liver. The surgeon can tell you more. Surgery is not a cure for biliary atresia. It won't work if the bile ducts inside your child's liver are damaged or missing. But, in many cases, it does help the liver work better for many years.

Building Your Child's Strength

Your child's surgery will likely take place shortly after the diagnosis of biliary atresia. To be sure your child is strong enough for surgery:

  • You may be asked to feed your child special formula and vitamins.
  • If you're breastfeeding, continue. Stop feedings only when your doctor tells you to.
  • Before surgery, a nasogastric (NG tube) may be placed to give your child food. This is a small, soft tube that is inserted into the child's nose. It travels down the esophagus and into the stomach. Food is then delivered through the tube directly into the stomach. If the NG tube is needed, the doctor will tell you more.

The Surgical Experience

  • Before surgery: Do not give your child anything to eat or drink for 4-6 hours before surgery. Follow all other instructions you are given.
  • During surgery: You will stay in a waiting room nearby during your child's surgery. Your child will be given medication to make him or her sleep during the surgery. How long the surgery will take depends on your child's condition.

Recovering After Surgery

  • Your child will stay in the hospital for 5 days or longer. During this time, the nursing staff will care for your child.
  • Your child will have frequent blood tests. These check the functioning of the liver.
  • Tubes will help drain fluid from around your child's incision. This helps the incision heal.
  • Your child won't be able to eat for 2-3 days after surgery, when the intestines begin working again. During this time, your child will be given nutrition through an IV (intravenous line). Once your child can eat again, he or she may be able to take food by mouth. Or, your child may be fed by NG tube for a time.
  • Your child may be given corticosteroid medication. This helps decrease inflammation (irritation and swelling) of the bile ducts in your child's liver. This medication may be given short-term or long-term.

Helping Your Child Heal After Surgery

Even after surgery, your child's liver won't be fully back to normal. The child will need special foods and medication to help the liver do its work. The following measures are most important as the child recovers from surgery. They may be stopped gradually as the child gets older.

  • Your child will need to be fed with breastmilk and/or special formulas. These provide types of fats that are easier for the liver to absorb.
  • Your child will take supplements that provide vitamins A, D, E, and K. These are fat-soluble vitamins that, without bile, your child's body can't absorb normally. Vitamin supplements may be taken long-term, or may be tapered off after a period of time.
  • Your child may take antibiotic medication for a few months after surgery. This helps prevent infection (cholangitis) caused by bacteria that travel from the intestines into the liver and bile ducts. Your child may be prone to infections in the liver and bile ducts, especially after a cold or other illness. These infections are also treated with antibiotic medication.
  • Your child will be given bile acid replacement medication. This helps improve bile flow. Your child may take this medication for several years.

Long-Term Follow-Up

Your child's health needs to be closely watched. At first, you will need to visit your child's pediatrician or liver doctor often. As your child gets older, you may be able to visit less often. These visits may include:

  • Blood tests to measure liver function and nutritional levels.
  • An ultrasound exam, which uses harmless sound waves to create an image of the liver.
  • Other tests as needed.
  • Immunizations and vaccinations against common childhood diseases. Make sure your child gets a flu shot every year. It's also important to get your child vaccinated against hepatitis A and B. These are liver diseases that can be much worse for children who have biliary atresia.

Looking to the Future: Preparing for a Possible Liver Transplant

Many children with biliary atresia need a liver transplant at some point. This may be needed within a few years after the Kasai procedure. Or, a transplant may not be needed for 10 years or more. Follow-up visits and tests help the doctor decide when a transplant is needed. The liver is an organ that can be donated by a living donor. This means another person might be able to donate a portion of his or her own liver. (The removed portion grows back.) If you want to know more about a living donor transplant, talk to your child's doctor.

When to Call the Doctor

After your child's surgery, call the doctor if you notice any of the following:

  • Worsening jaundice, or jaundice that comes back after treatment
  • Itching
  • Abdominal swelling or pain
  • Poor growth
  • Any signs of a vitamin deficiency, such as poor balance, inability to walk, bowlegs, or scaly skin
  • Bloody stool or vomit
  • Pale or white-colored stools
  • No bowel movement or watery bowel movements for more than 3 days
  • Confusion, delirium, extreme sleepiness, or loss of consciousness
  • Fever over 100.4?F
  • Unusual bruising or bleeding
  • Redness, swelling, or drainage from wounds