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Biliary atresia

Definition

Biliary atresia is an obstruction of the bile ducts caused by their failure to develop normally in the fetus. This is a congenital condition (present at birth).
Causes, incidence, and risk factors    Return to top

Biliary atresia is caused by the abnormal 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 babies with biliary atresia, 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, will eventually be fatal.

Newborns with this condition may appear normal at birth but 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.

Symptoms

jaundice, a yellow color of the skin and eyes
may be present at birth or develop over several months
may be progressive or intermittent
slow or absent weight gain
slow growth in a child 0-5 years old
irritability
dark urine
splenomegaly (enlarged spleen)
stools - pale or clay colored
stools - floating
stools - foul smelling

Signs and tests

During a physical examination, the doctor may detect an enlarged liver.

Tests that reveal biliary atresia include:

increased bilirubin in the blood
abdominal x-ray shows an enlarged liver and spleen
abdominal ultrasound
HIDA scan (nuclear test to determine bile flow)
cholangiogram to determine patency of ducts
liver biopsy to determine degree of cirrhosis or to rule out other causes of jaundice
Treatment    Return to top

An operation called the Kasai procedure is done to connect the liver to the small intestine, bypassing the malformed ducts. It is most successful if performed before the baby is 10 to 12 weeks old. However, a liver transplant may still ultimately be required.

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

liver failure
infection
surgical complications
failure of the Kasai procedure
irreversible cirrhosis
Calling your health care provider    Return to top

Call your health care provider if your child appears jaundiced, or if other symptoms suggestive of this disorder develop.


Update Date: 1/12/2003

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites.

Page last updated: 06 July 2004

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