By: Pamela L. Valentino, MD, MSc, FAAP & Sanjiv Harpavat, MD, PhD, FAAP
Biliary atresia, a serious liver disease that affects infants, occurs when tubes called bile ducts become blocked. Normally, bile ducts carry a liquid called "bile" from the liver to the intestines to help us digest our food.
With biliary atresia, the blocked bile ducts do not allow bile to pass. The bile cannot reach the intestines. Instead, bile backs-up in the liver and causes damage.
What is a Kasai portoenterostomy?
The Kasai portoenterostomy is the key treatment for biliary atresia. This operation has two steps:
First, surgeons remove all the bile ducts outside the liver, including the blocked bile duct.
Then, surgeons connect the liver directly to the intestines. In this way, the liver may be able to drain bile directly to the intestine.
To perform the Kasai operation, the surgeon makes an incision below the ribs on the right side of the abdomen.
What happens before surgery?
Your baby will have to fast before surgery. During this period, they receive intravenous (IV) fluids with sugar to maintain hydration and nutrition.
How do I care for my child after biliary atresia surgery?
Infants can go home after they can tolerate feedings again. This usually is 4-7 days after the operation.
There are usually 3-4 medications to go home with the baby. These include supplements for vitamins A, D, E and K, antibiotics and ursodiol. Ursodiol is a medication that is thought to help bile flow to the intestines.
Infants should return to the hospital if they stop eating, appear weak, have fewer wet diapers than usual or develop a fever.
What are possible complications with biliary atresia surgery?
Infants can develop "cholangitis." This is thought to be an infection of the new connection between the liver and intestines.
Cholangitis typically causes fever and an increase in conjugated bilirubin levels. It is treated with antibiotics, typically by IV in the hospital for infants.
Many infants also develop liver scarring over time. This scarring is referred to as "fibrosis" or "cirrhosis." If scarring progresses, problems such as ascites or vomiting blood can develop. In these instances, a liver transplant may eventually be needed.
When will my child need to see the doctor again?
At the time of discharge from the hospital, your doctor will discuss a follow up plan with your family. Commonly, an office visit is scheduled within two weeks after discharge from the hospital. Your child should also have follow-up visits with their pediatric surgeon and pediatric gastroenterologist.
During the first years after surgery, visits to the doctor might be very frequent to monitor liver function along with growth and development.
What is the long-term outlook after surgery?
The outlook following a Kasai operation for the treatment of biliary atresia has been well studied. Most children will require close follow up by their pediatrician, who will closely monitor their growth and development.
Can the Kasai operation help prevent the need for liver transplant?
Biliary atresia is the most common cause of liver transplantation in children. About one-third of all children undergoing a Kasai operation will not need a liver transplant. In contrast, two-thirds of children who have had a Kasai operation will ultimately need a liver transplant.
However, babies who are identified with and treated for biliary atresia before they reach 1 month old have a better chance at improved outcomes and are at lower risk of receiving a liver transplant. (For more information, see "Identifying Babies With Biliary Atresia Early: AAP Guidance.")
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About Dr. Valentino
Pamela L. Valentino, MD, MSc, FAAP, a member of the American Academy of Pediatrics (AAP) Section on Gastroenterology, Hepatology and Nutrition, is the clinical center director of Hepatology and medical director of liver transplant at Seattle Children’s Hospital. Dr. Valentino is also an Associate Professor at the University of Washington in the Department of Pediatrics. It is Dr. Valentino’s mission to help children with liver diseases and liver transplants lead health full lives. She focuses her clinical work and research efforts on bile duct diseases, bile duct complications following liver transplant and Wilson disease.
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About Dr. Harpavat
Sanjiv Harpavat, MD, PhD, FAAP, is lead author of the AAP clinical report, "Identifying Infants with Biliary Atresia by 2-4 Weeks of Life: Guidance for the Primary Care Provider" and Chair of the AAP Section on Gastroenterology, Hepatology and Nutrition.
Editor's note: The authors would like to acknowledge David T. Schindel, MD, FAAP, and Humberto Lugo-Vicente, MD, for writing a previous version of this article. |