Biliary Atresia

What is biliary atresia?

Biliary atresia begins in early infancy and causes damage to the liver due to the destruction of ducts which carry bile from the liver to the small intestine. Early damage mainly affects the major bile duct outside the liver which is why this is also referred to as extra hepatic biliary atresia. It is a relatively rare disease affecting about one in every 10,000 to 20,000 infants. This prevents bile from leaving the liver and it is accumulated there causing progressive damage to the liver tissue. Also, there is ongoing damage to the smaller bile ducts inside the liver. Unless bile flow can be established, liver function is gradually lost. The cause of biliary atresia is not known. It is not contagious, so it cannot be passed from one child to another. A genetic cause has not been found, nor can it be attributed to any specific aspect of prenatal care.

Symptoms:

Biliary atresia is commonly diagnosed within the first two months of life. Common signs include:
  • Newborn jaundice that does not go away by four weeks of age 
  • Dark urine and pale, clay–colored stools 
  • Enlarged liver, spleen and or swollen abdomen

How is it treated?

It is important that bile flow be established. Otherwise, the liver will suffer more damage resulting in liver failure. The most common treatment is an operation called a Kasai procedure. The operation involves removing the damaged duct outside the liver, and replaces it with a new drainage system created from a piece of the small intestine. This may partially or fully alleviate the jaundice, but it will not reverse the liver damage that has already occurred or prevent any ongoing damage. The Kasai procedure is successful in a third to half of all patients. If jaundice is fully relieved, most children will grow and develop normally, although cirrhosis or scarring of the liver generally occurs despite a successful Kasai procedure.

What will happen in the hospital?

Your child will either be in the hospital already, or you will be given information on what time to arrive for your child’s procedure the day before. There will be accommodations on one of the pediatric units where parents will be able to room in with their child over the course of the hospital stay. Plan to stay for about 5-7 days (this may vary with each child). The operating room is located on the 3rd floor of the West Pavilion Children’s Hospital. Before the procedure begins you will speak with the recovery room nurses, the anesthesia doctors, and your child’s surgeon(s). During the procedure, parents are able to wait in the waiting room or in the room where their child will return after the procedure. The 7th or 8th floor is where most children go after their procedure, in some situations the pediatric intensive care unit may be warranted for closer monitoring of your child. You will be made aware of this once your child is in the recovery room.

Operation

One particular type of operation, the Kasai Procedure, (also called a portoenterostomy), can be done to help re-establish bile flow from the liver to the intestine. This operation involves connecting the liver directly to the small intestine with a loop of the small intestine. This bypasses the blocked ducts, letting bile flow from the small bile ducts inside the liver into the intestine. The surgery is 3 hours long in general, and your child will have an incision to his/her upper abdomen. A tube will also be placed through your child’s nose, into the esophagus, and into the stomach in order to decompress the abdomen and remove gastric secretions. Pain will be well controlled with intravenous medication initially, and then your child will be able to take pain medications by mouth once they are able to tolerate fluids.

An early operation can improve the outcome of biliary atresia. Twenty-five percent of infants will have good bile flow after surgery while 50% will have some bile flow. The remaining 25% will have little or no bile flow and will require early liver transplantation. Liver transplantation is the only known cure for biliary atresia and most children will likely need a liver transplant during their childhood or early adult life.

When will my child be discharged?

Generally, your child will stay in the hospital for 5 -7 days:
  • On postoperative days 1-2 your child will not be able to eat or drink anything and will receive intravenous hydration. The nasogastric tube will remain in your child’s nose. 
  • On postoperative days 2-3 your child may start to drink Pedialyte and if they are able to tolerate that without any vomiting, their diet will advance to breast milk or formula (the nasogastric tube may be removed at some point during this time). 
  • On postoperative days 4-5 your child should be able to tolerate full feedings of breast milk or formula, and they must have a bowel movement before they are able to go home.
In addition, they must not be having any fevers and their pain must be well controlled with medications by mouth.

What will be my child's recovery?

In the early post-operative period, yellow or green colored stools and a declining bilirubin (level measured in the blood) are evidence of bile flow from the liver. However, there is still a risk of liver disease progression and close follow up is mandatory. One half to two thirds of all patients with biliary atresia will develop progressive liver disease.

What should I be looking out for after the operation/hospitalization?

Cholangitis - An inflammation of the bile ducts in children with biliary atresia. This is a known problem after the Kasai procedure that occurs infrequently. Symptoms include irritability, fever, jaundice and poor appetite. Medical treatment should be sought immediately to treat the infection and prevent further liver damage. Cholangitis is treated with intravenous antibiotics in the hospital, and occasional need for steroids.

Failure to Thrive
- Lack of bile salts in the intestine, which are needed for fat digestion, result in poor growth and fat-soluble vitamin deficiency. Vitamins A, D, E and K can be given orally to prevent deficiencies of these vitamins. Special infant formulas are usually needed to help with growth.

Cirrhosis - Scarring to the liver occurs causing irreversible damage. Once cirrhosis develops, the functions of the liver start to be interrupted and liver transplantation is considered.

Portal Hypertension - As the liver becomes scarred, veins travelling through the liver become constricted. This impairs the flow of blood and increases the pressure in these veins, specifically in the portal vein (a major vein of the liver system) leading to a big spleen and fluid problems (such as ascites).

Ascites – Ascites is a complication of portal hypertension. This occurs when there is a buildup of fluid in the space between the lining of the abdominal wall and the lining of the organs.

Varices - Another complication of portal hypertension is varices. Varices occur when the increased pressure causes blood to flow through smaller veins to the stomach then into the esophagus which lead them to weaken and swell. Varices can lead to bleeding. This bleeding can cause dark colored stools and blood-stained vomit. Immediate medical attention is necessary if this occurs.

What is the success rate after my child's procedure?

The success rate for portoenterostomy can increase with a surgeon’s experience and a post-operative management plan. This may or may not include steroids to decrease inflammation, ursodeoxycolic acid to stimulate bile flow, and antibiotics to decrease the incidence of cholangitis. Patients also receive fat soluble vitamins (A, D, E and K).

Once you have been discharged home, the pediatric surgeon will see you within 2 weeks to evaluate the incision and monitor your child’s progress after the surgery. You can call the pediatric surgery office at 203-785-2701 if you have any questions or concerns after surgery.