Biliary atresia

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 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 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 15,000 live births.

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
growth, slow (child 0-5 years)
irritability
stools, pale or clay colored
dark urine
splenomegaly (enlarged spleen)
stools - floating
stools - foul smelling

Signs and tests:

Physical examination shows 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:

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 eight weeks old. It is usually only a temporary solution and a liver transplant may 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:

Call for an appointment with your health care provider if your child appears jaundiced, or if other symptoms of this disorder develop.

Prevention:

There is no proven way to prevent this disorder.

Review Date: 5/21/2001
Reviewed By: Elizabeth Hait, M.D., Department of Pediatrics, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, OH. Review provided by VeriMed Healthcare Network.

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