What is Biliary Atresia?

Is a life-threatening condition in infants. Is a congenital disease of the liver and one of the principal forms of chronic rejection of a transplanted liver allograft. Occurs when the bile ducts inside or outside the liver is blocked or absent. The bile ducts help to remove waste from the liver and carry salts that help the small intestine digest fat and absorb the fat soluble vitamins A, D, E, K. as a birth defect, bile flow from the liver to the gallbladder is blocked. This can lead to liver damage and cirrhosis of the liver, which can be deadly.

What are the Symtoms?

  • Jaundice, when the skin and whites of the eyes turn yellow.
  • Dark Urine (tea color).
  • Pale or white stools, between 2 – 6 weeks of birth.
  • Slow weight gain and growth.

How is BA treated?

Biliary atresia cannot be treated with medication.

A Kasai procedure or hepatoportoenterostomy is done. The Kasai procedure is an operation to re-establish bile flow from the liver into the intestine. It is named after the surgeon who developed it.

Success with the Kasai procedure is related to:

The younger an infant at the time of surgery, the more likely the surgery will be successful. By the time an infant is 4 months old, surgery is unlikely to be helpful

Extent of liver damage (cirrhosis) at the time of surgery.

The number and size of microscopic ducts in the scarred tissue that can drain bile.

The experience of the surgical and medical team. Centers with teams made up of specialists with extensive experience have success rates that are greater than those centers with less experienced teams

Liver Transplant

If there is still not enough bile flow with the Kasai procedure, liver transplantation is a final option. A liver transplant operation removes the damaged liver and replaces it with a new liver from a donor. After a transplant, ongoing lifelong care is required. Frequent contact with physicians and other members of the transplant team is also necessary.