Biliary Atresia

Definition:  An obstruction of the bile ducts caused by their failure to develop normally before birth (in utero).
Causes, incidence, and risk factors:
  Biliary atresia is caused by the abnormal and inadequate development of the bile ducts inside or outside the liver. The purpose of the biliary system is to remove waste products from the liver, and to carry bile salts necessary for fat digestion to the small intestine. In this condition, bile flow from the liver to the gallbladder is blocked. This can lead to liver damage and cirrhosis of the liver, which, if not treated, is eventually fatal. 

Newborns with this condition may appear normal at birth but persistent jaundice develops by the 2nd or 3rd week of life. The infant may gain weight normally for the 1st month, then weight loss and irritability develop accompanied by increasing levels of jaundice. It is not known why the biliary system fails to develop normally.  The incidence of biliary atresia is approximately 1 out of 20,000 live births.

Symptoms:  Jaundice, a yellow color of the skin, may be present at birth or develop over several months, may be progressive or intermittent, slow or absent weight gain, slow growth (child 0-5 years),  irritability, stools are pale or clay colored, splenomegaly, stools - floating, stools - foul smelling

Signs and tests: Physical examination shows an enlarged liver. Tests that reveal biliary atresia include: abdominal x-ray shows an enlarged liver and spleen, abdominal ultrasound,  cholangiogram to determine patency of ducts, liver biopsy to determine degree of cirrhosis or to rule out other causes of jaundice.

Treatment:  Surgery is usually performed within the first 2 months of life. The surgery connects the liver to the small intestine, bypassing the malformed ducts. A liver transplant may also be recommended to treat certain types of biliary atresia.

Expectations (prognosis):  Early surgical intervention will improve the survival of more than one third of those affected by the condition. The long-term benefit of liver transplant is not yet known, but is expected to improve survival.

Complications:  irreversible cirrhosis. As with any procedure we perform, your child's safety and well-being our #1 priority.  As always, feel free to contact us with any question that you have.  We would be happy to answer it for you.