By: David T. Schindel, MD FAAP & Humberto Lugo-Vincente, MD
Biliary atresia is a condition that affects a baby's bile ducts, tubes that carry digestive fluid from the liver to the intestines. The bile ducts may be too narrow, blocked or missing. Because of this, bile builds up in the liver and causes damage.
What is a Kasai operation?
If your child's doctor suspects they may have biliary atresia, imaging studies can be helpful. However, surgery with examination of the bile ducts is the gold standard of diagnosis. A Kasai operation is a type of surgery to remove the diseased bile ducts to the base of the liver and re-establish bile flow from the liver using a portion of the child's intestine.
How is biliary atresia surgery performed in children?
Either using an incision made below the ribs on the right of the abdomen or a laparoscopic technique, the bile ducts are first inspected. A study called a cholangiogram is performed to confirm the diagnosis of biliary atresia.
To perform a cholangiogram, the surgeon places a small tube into the
gallbladder, if present. Then they inject a medication that can help find the bile ducts using an X-ray machine.
If the gallbladder and bile ducts are not open and bile cannot drain, the child has biliary atresia. The surgeon will proceed to remove the very small or abnormal gallbladder and abnormal bile ducts where they connect with the liver. A piece of small intestine called the jejunum is then used to replace the bile ducts, making a connection between the liver and the intestine. This connection will hopefully re-establish bile drainage into the intestine, reversing the effects of the disease.
What happens before surgery?
To prepare your child for surgery, they will get intravenous (IV) fluids to maintain hydration, sugar levels and nutrition. In addition, they'll be given IV antibiotics are provided to reduce infection. Blood products, such as red blood cells or platelets, might be needed during surgery..
When can my child go home after surgery?
It is common for children having a Kasai operation to be admitted to the Intensive Care Unit (ICU) for recovery after surgery. Your child will be watched very closely for signs of bleeding and proof that bile is successfully draining from the liver into the intestine. Signs this is happening include the return of bile pigment (greenish color) to the stools as well as improvement in the baby's liver function tests.
Once your child's intestine function returns, they can start feeding again. Once their incision is healing properly, their liver functions are getting better, and your baby is tolerating a proper diet, the surgeon will likely then determine they are ready to go home.
How do I care for my child after biliary atresia surgery?
Unless there was a complication, your child's incision can be cleaned as you would the rest of their body. If your baby takes formula, there may be some changes your child's health care team will recommend after surgery. Some medicines that your child might need include steroids to reduce inflammation, oral antibiotics to avoid bile duct infections, and vitamins supplements. If your child stops eating, feels weak, has fewer wet diapers or develops a fever, they should return to the hospital.
What are possible complications with biliary atresia surgery?
Complications may be divided into those occurring soon after the operation versus those occurring later. The most common early complication is a wound infection at the site of the incision. These infections can be treated with oral antibiotics alone but may also need more care to heal.
Another type of infection that can occur at any point after a Kasai operation is called cholangitis. When bile flows through the new intestinal connection between the liver and bowel, a bile duct infection known as cholangitis can develop. Cholangitis typically causes fever and even the return of
jaundice. IV antibiotics are typically needed to treat cholangitis.
Over time scarring and abnormal bile drainage from the liver can cause progressive liver damage and more scarring of the liver. This scarring is referred to as hepatic fibrosis. If the scarring progresses, liver failure can result and create the need for a hepatic transplant. Biliary atresia is the most common cause of liver transplantation in children.
When will my child need to see the doctor again?
At the time of discharge from the hospital, the surgeon will discuss a follow up plan with your family. Commonly, an office visit is scheduled 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. If your child is doing well after surgery, medication needs may be limited to only a daily multivitamin. About one-third of all children undergoing a Kasai operation will be long-term survivors. Unfortunately, two-thirds of children who've had a Kasai operation will ultimately need a liver transplant to resolve the effects of ongoing liver damage that result from the scarred bile ducts and liver.
About Dr. Schindel
David T. Schindel, MD, FAAP, a pediatric surgeon with Pediatric Surgical Associates, works out of Medical City Children’s Hospital in Dallas. He is a member of the American Academy of Pediatrics Section on Surgery and the American Pediatric Surgery Association. Dr. Schindel's clinical and research interests include congenital surgery of the newborn, such as biliary atresia, choledochal cysts, abdominal wall defects, lung malformations and tracheoesophageal surgery. He also has significant interest and experience in the surgical management of pediatric Inflammatory Bowel Disease.
About Dr. Lugo-Vicente
Humberto Lugo-Vicente is Professor of Pediatric Surgery at the University of Puerto Rico, Universidad Central del Caribe, and Ponce Schools of Medicine. He is the Director of Pediatric Surgery at San Jorge Children's Hospital and the chief editor of Pediatric Surgery Update (http://pedsurgeryupdate.com/).