What is this disease?

• Biliary atresia is a condition involving bile ducts, either because the bile ducts developing abnormally or because of bile ducts becoming inflamed or obstructed. It eventually leads to a complete blockage of bile flow from the liver. This in turn causes scarring (fibrosis) in the liver.

How it is diagnosed?

• Prolonged jaundice (jaundice lasting more than two weeks in a full term baby or three weeks in a pre-term baby) along with pale white stool are initial findings which raises suspicion about this disease. A number of investigations will be needed to confirm the diagnosis. They include Blood and urine  tests, an ultrasound, Liver excretion test like HIDA scan  and  a liver biopsy. Some may need  a small operation(intraoperative cholangiogram) to confirm or rule out a diagnosis.

How it is treated?

• Surgical management is needed in these children. It starts with an  operative cholangiogram, whenever indicated , followed by  “Kasai procedure”(portoenterostomy). The aim of the procedure is to help bile drain from the liver into the gut

When it should be operated?

• If a baby has not had a Kasai procedure in the first two to three months of life, the success rate of the operation is very low. It is generally accepted that one third will have successful Kasai for life, one third will have successful Kasai for 10-20 years and one third will have failed Kasai, necessating liver transplant.

Are there other alternative methods of treatment?

• Liver Transplant can be considered in a child who had presented after 3 to 4 months and have very high chances of failure of Kasai procedure.

What all I need to know before my child surgery?

• Read “All you need to know before your child’s surgery” information booklet in website

How is the surgery done?

• The aim of the Kasai  operation is to make a drainage tube to allow bile to drain from the live.  During the operation the gallbladder and all the abnormal bile ducts outside the liver are removed. At the highest point, along the surface of the liver, there are usually enough very small bile ducts which will allow some bile flow to be re-established. a loop of intestine in joined  to the under surface of the liver.

Remarks

• For more details of surgery, contact your surgeon.

Related Photographs and videos

• Few photographs of steps done by me are given here for learning purposes.