- Pediatric Surgery
- Biliary Atresia
Biliary Atresia
Biliary atresia is a rare but severe liver condition that affects infants. Cells in the liver produce bile, a liquid that helps digest fat. It also carries waste products from the liver to the intestines for removal.
In biliary atresia, these bile ducts are blocked. When the bile ducts don’t work properly, the buildup causes scarring and damage to the liver. This can eventually lead to liver failure if not properly treated.
Biliary atresia occurs in one in 15,000 babies.
What causes it?
The cause of biliary atresia is unknown. The condition develops during the neonatal period. In 10-15% of children, biliary atresia is associated with a syndrome that may include anomalies of the spleen, intestine, heart, or urinary system.
What are the symptoms?
Babies with biliary atresia appear healthy when they are born. Signs of the condition usually appear anytime in the first two months after birth. This can include:
- Jaundice lasting longer than two weeks
- Dark-colored urine
- Pale or clay-colored stools
- Swollen belly
- Weight loss and increased irritability
How is it diagnosed?
Several tests are needed to confirm biliary atresia accurately and confirm how it will be treated. Your child may undergo blood tests, abdominal ultrasound, liver biopsy, and a special X-ray called operative cholangiogram to look at the bile ducts.
How is it treated
Biliary atresia can’t be treated with medication. A unique surgery called a Kasai procedure (also known as a hepatoportoenterostomy) is performed to create a path of bile flow from the liver into the intestine. The Kasai procedure is not a cure for biliary atresia, but it does allow babies to grow and enjoy a higher quality of life for many years. In the most severe cases, a liver transplant may be needed.
Long-term Treatment
Even with a successful Kasai surgery, approximately 80% of patients with biliary atresia will need a liver transplant by the age of 20 years old. Children with biliary atresia are followed closely to monitor their nutrition and liver function.
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