What is Biliary Atresia?

Biliary Atresia (or BA) is a congenital defect involving the liver and gallbladder. This condition is caused when the duct that carries bile from the liver to the gallbladder becomes blocked. It is a rare condition, occurring only once in 15,000 to 20,000 births, more commonly among girls and those of Asian or African-American descent.

Bile is the fluid in your body that breaks down fats into fatty acids, aiding in the digestive process. Bile is made by your liver and stored in the gallbladder.

What causes Biliary Atresia?

Since BA is a congenital defect, it is not currently known what causes this disease to occur. Fetal biliary atresia is developed during pregnancy and is present at birth. A second type, called perinatal Biliary Atresia, develops in infants 2-4 weeks old. Biliary atresia is not hereditary; even with twins, only one may form the disease and the other not.

Biliary atresia has NOT been linked to any action or inaction during pregnancy.

 

Symptoms

Newborns with biliary atresia appear normal at birth. The first sign of biliary atresia is jaundice (yellowing of the skin); if your infant is still jaundiced two weeks after birth, you should talk to your doctor about liver tests. Other symptoms of biliary atresia can include darkened urine, lightened stools, a distended abdomen, or weight loss.

Stools light in color (pale grey or white) after two weeks of age should be discussed immediately with your child’s doctor, as they are a definitive sign of liver problems.

 Diagnosis Of Biliary Atresia:

Several tests can be done to properly diagnose biliary atresia.

Blood tests, including liver enzymes, bilirubin levels, albumin and total protein levels, check the functionality of the liver.

Radiologic tests include:

                X-Rays: check for an enlarged spleen or liver

                Ultrasound: Checking for the gallbladder

Nuclear test: HIDA (Hepatobiliary iminodiacetic acid) scan, also called a cholescintigraphy, checks the functionality of the ducts and the gallbladder

Biopsy: Either a needle biopsy or tissue biopsy can be done. A tissue biopsy would be performed as part of surgery.

Diagnostic Surgery: Confirms a biliary atresia diagnosis. During the operation, dye will be injected to check if the flow of bile is correct.

Biliary Atresia Treatment:

There is currently no medication that will treat biliary atresia. Medications cannot unblock the ducts, nor grow ducts where none were present. There are two surgical options for treating biliary atresia:


Kasai portoenterostomy: This operation will correct the flow of bile from the liver to the gallbladder using a piece of the infant’s intestine. This procedure can improve the life of the patient, but is not a cure. Without this surgery, the child has a very poor prognosis and may not live past the age of two. Children who undergo this procedure can go on to live without any major liver problems or further jaundice.

Liver Transplant: A transplant is necessary if the Kasai procedure is unsuccessful, or the liver has been too damaged due to the biliary atresia or other liver issues.

Due to recent developments in medicine, liver transplants have become highly successful in treating biliary atresia. In the past, only livers from children could be used for transplantation into infants; presently, adult donors can donate a part of their liver to a patient. Because living cells grow quickly, both donor and receiver can grow full, healthy livers. 

Related Resource Pages on Band Back Together:

Birth Defects

Liver Disease

Transplant

Other Biliary Atresia Resources:

PubMed Health – easy to understand explanations of diseases

National Institute of Diabetes and Digestive and Kidney Diseases – conducts and supports research

Medline Plus - National Institute of Health site for understand diseases

Cincinnati Children’s Hospital - research

Children’s Memorial Hospital - research

 

Support

Liver Families - support for families affected by biliary atresia, or any other pediatric liver disease or transplant

Biliary Atresia Network - a Yahoo! Group for families

C.L.A.S.S. - Children’s Liver Association for Support Services – addressing emotional, educational and financial needs of pediatric liver cases

American Liver Foundation - “facilitate, advocate and promote education, support and research for the prevention, treatment and cure of liver disease.”

Children’s Liver Disease Foundation - support and research in the UK for children affected by liver diseases