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Biliary Atresia
What is biliary atresia?
Biliary atresia is a condition in which the ducts that connect the liver to the intestine and the gall bladder become scarred and blocked. One of the things that the liver does is make bile. The bile flows to the intestine through these ducts where it helps digest food. The bile blocked in the liver causes scarring in the liver called cirrhosis.
The bile trapped in the liver also backs up into the blood and causes the skin and eyes to look yellow. This is called jaundice.
Who gets biliary atresia?
Biliary atresia is a rare condition that is suspected in infants that have jaundice after 2 weeks of age. Since bile in the intestine is what give bowel movements color (yellow, green or brownish), they will look very pale. The word for this is acholic.
How is biliary atresia diagnosed?
Once biliary atresia is suspected because of the jaundice, the baby will have blood tests, an abdominal ultrasound, a scan (a special kind of x-ray), and a liver biopsy. The results of all of these tests together will help the doctors decide if the baby has biliary atresia. These tests will be scheduled as quickly as possible because the results of the surgery are better for babies who are less than 70 days old.
What is the treatment for biliary atresia?
There is no treatment for biliary atresia except for an operation called the Kasai procedure. The purpose of the surgery is to replace the scarred ducts. The surgeon will make a large incision on the belly, take out the scarred ducts and make a way to drain bile using the baby’s own intestine.
What can I expect from surgery?
After surgery, there will be a tube in his/her nose going to the stomach to drain fluid and prevent vomiting. The baby will not be able to eat for several days while the intestines heal. The baby will get sugar water through an intravenous (a very small tube that goes into one of the baby’s veins).
The intravenous is also a way for the nurse to give pain medication. It usually takes 3 or 4 days for the baby to be well enough to have the tube removed and be able to eat. In addition to having a bandage over the large incision, therewill be a small tube called a drain that lets fluid out of the belly. This will be taken out before the baby goes home. The color of the baby’s bowel movements will change to green/yellow if the surgery is successful in getting bile from the liver to the intestines. This may take up to 2 weeks but often happens while the baby is still in the hospital.
What should I bring to the hospital?
The hospital has everything needed to take care of a baby but if your baby prefers a special bottle, nipple, or uses a pacifier, please bring it. You may bring clothes, blankets or little toys.
When will my child be able to go home?
Your baby will be in the hospital for 5 to 7 days.
What care is needed at home after surgery?
- Care of the incision: The dressing will be off and no special care will be needed.
- Bathing: Sponge bathing until 1 week after surgery, then you may give your baby a tub bath.
- Medications: You will get a prescription for pain medication and instructions on when to give it. Your baby will have to take several other medications for a long time--a year or longer. These are to encourage bile flow and prevent infection. You will be given prescriptions & instructions. It is very important to follow the instructions carefully.
- Diet: If you are breastfeeding, the nurses will help you pump and save your milk during the time that your baby can not eat. If you are not breast feeding, your baby will need to have a special formula that is easier to digest than regular formula.
When should I call the surgery team prior to the postoperative checkup?
Call for fever above 100.5 F, drainage from the incision, vomiting, pain not relieved by medication, or stools turning white again for more than a day.
What should I call my pediatrician for, and when should we see him/her?
The surgeon will send a letter to your pediatrician about the surgery if there are changes in usual pediatric care such as immunizations, advancing feedings, etc.
What are the long term consequences?
The surgeon and/or gastroenterologist have explained that biliary atresia is a serious and complex problem. Along with the pediatrician, they will follow your baby carefully. Even after a Kasai procedure, about 50-60% of babies will require a liver transplant at some time in their life in order to survive.
Please, reproduce and distribute this sheet to your surgery families. This teaching sheet can also be downloaded at www.APSNA.org.
Copyright 2006, Laura Flanigan,. Copied with permission by Jones and Bartlett Publishers, Sudbury, MA."