Overview of Biliary Atresia:

biliarySystemBiliary Atresia is a rare but serious liver disorder which affects infants and first becomes evident within the first 6 or so weeks of life of the child. Biliary Atresia, referred to as “BA”, tends to affect girls more often than boys and is only present in about 1 out of every 15,000 children in the United States.

A liver produces a liquid called bile, bile carries waste products from the liver as well as helping to digest fat. Bile travels through a system called The Biliary System.
This Biliary System is the system of ducts that drain bile from the liver and allow bile to drain from the liver to the intestines.

biliaryBiliary Atresia is either a blockage or complete lack of the bile duct which drains bile from the infant’s liver which prevents bile flow from the liver to the gallbladder and causes bile to build up inside the liver. Bile build-up within the liver causes damage to the liver tissue, liver cell failure (known as Cirrhosis), and finally results in complete liver failure.

Currently there is no known cause for Biliary Atresia, Biliary Atresia however, is not an inherited condition and there is no known connection with medication taken during pregnancy and Biliary Atresia.

Symptoms of Biliary Atresia:

The symptoms of Biliary Atresia typically first begin to manifest themselves within a week or two of birth (in many cases the baby appears to be completely healthy at birth). The first symptom is usually jaundice which typically presents itself in the first 1-6 weeks after birth.

Jaundice causes yellowing of the skin as well as the whites of the eyes, this jaundice is caused by the liver not removing bilirubin from the blood, this bilirubin is a yellow substance, hence the yellowing. Jaundice is fairly common amongst infants, however if your child continues to show signs of jaundice beyond 2 weeks of age it’s a good idea to get it checked out by your doctor and check for liver disease.

Other than jaundice the symptoms include dark urine, Acholic stools (clay-colored stools), lightened colored stools (pale, grey, or even white), a swollen abdomen (noticeably swollen), and a hardened liver. If your infant seems to be affected by any of these symptoms beyond two weeks old get it checked out by your doctor and check for liver disease.

Biliary Atresia Treatment:

It is important for you to know that Biliary Atresia is not treated with medication. If it appears that Biliary Atresia is the cause of the jaundice in your infant there are several different form of diagnosis ranging from blood tests to a liver biopsy(removing a piece of the liver’s tissue for further testing).

If your infant is diagnosed with Biliary Atresia the surgeon will typically go ahead and perform the Kasai procedure(Hepato-portoenterostomy) which is a surgical procedure in which the surgeon removes the blocked bile ducts outside of the liver and replaces them with a piece of the infant’s own intestines which allows the bile to drain directly to the intestines.

The Kasai procedure is typically successful in about 70% of the attempts to perform the procedure. Success is determined by the bile draining from the liver, which, in turn, allows the jaundice level to go down. Note: The Kasai procedure is typically not a cure for Biliary Atresia(however there have been some cases where a child has grown and lived jaundice free and free of liver problems after a successful Kasai), it merely allows the infant to live fairly healthily for several years.

If the Kasai procedure is not successful typically liver disease condition worsens and liver transplantation is required within the first couple years of life.

That brings us to liver transplantation, liver transplantation is a very successful form of treatment for Biliary Atresia and in the past several years the success rate has gone up and the risk involved has plummeted. Infants who have a successful transplant are living into adulthood and even having their own children.

Thanks to modern medicine children are now able to receive either a whole liver from another child or more recently the child can receive a partial liver from a living donor. Healthy liver tissue grows very quickly and over a short period of time both the donor’s and recipient’s livers grow to normal size. Because of the possibilities in partial liver donation nearly every child with Biliary Atresia will receive the liver he or she needs to continue life, a truly amazing testament to the marvels of modern medicine.

Links & Continued Research: