Biliary atresia is a condition that causes blockage of the bile ducts in newborn babies. This is a rare condition that appears in approximately 12,000 births each year in the United States.
Bile is a substance created in the liver that helps digestion. It flows from the liver through a system of tubes (bile ducts) to the small intestine. For children who have biliary atresia, the bile ducts between the liver and the small intestine are blocked by scar tissue. This causes bile to remain in the liver, causing organ damage.
Babies with biliary atresia can often be treated with a surgical procedure. However, the majority of patients will still need a liver transplant at some point during their childhood or early adulthood.
The cause of biliary atresia is unknown. The condition develops during the neonatal period, and most of the time it is not hereditary and is not associated with other abnormalities. In 10 percent to 15 percent of infants, biliary atresia can be associated with other abnormalities of the spleen, intestines, heart or urinary system.
Many newborns with biliary atresia appear healthy when they are born. Symptoms may not begin until the second or third week of life.
The first symptoms are usually jaundice and abnormal-colored stools. Jaundice occurs when too much bilirubin builds up in the blood, causing the skin and whites of the eyes to turn a yellowish color. Many healthy newborns experience jaundice during the first week of life, and without other symptoms it usually does not indicate biliary atresia. Normal stool color for newborns is yellow or greenish brown. With biliary atresia, stool is a light clay/gray/tan color.
Babies with biliary atresia may show these symptoms:
In addition to the routine physical exam all newborns undergo at birth, doctors may recommend any of the following tests if biliary atresia is suspected.
Checking the bilirubin level in the blood can help make a diagnosis. There are two types of bilirubin: direct bilirubin and indirect bilirubin. Babies with biliary atresia have high levels of direct bilirubin. This can usually be detected before any symptoms are present. Babies whose results indicate high levels of direct bilirubin can be referred to a specialist for a thorough examination and prompt treatment if needed.
High-frequency sound waves are transmitted through the body, creating images of the baby’s internal organs. An abdominal ultrasound can detect abnormalities with the gallbladder. Babies with biliary atresia often have a gallbladder that is small or collapsed.
A small amount of tissue is removed from the liver to examine for abnormalities. The procedure can be done in the operating room or with interventional radiology. The liver biopsy can help the pathologist look for other causes of liver failure or determine if the scarring around the small bile tubes in the liver is consistent with biliary atresia.
This test is an X-ray of the bile ducts. Contrast dye is injected to see how well bile is flowing through the baby’s system and if blockages are present.
Also called cholescintigraphy, an HIDA scan allows the doctor to see the bile ducts and gallbladder to determine if bile is flowing effectively.
There is no cure for biliary atresia, but it can be treated to improve symptoms. The goal is to diagnose and begin treatment for biliary atresia as quickly as possible; the earlier the diagnosis, the more treatment options available.
A procedure called Kasai surgery can be performed to manage symptoms and delay progression of the disease. This procedure is more effective in the earlier stages of disease.
The damaged bile ducts between the liver and the small intestine are removed, and a portion of the small intestine is connected directly to the liver. Without the clogged bile ducts, bile can flow effectively from the liver into the small intestine. Kasai surgery has a high success rate. However, if the procedure is not successful, the child will probably need a liver transplant.
When the liver is damaged and begins to fail, a liver transplant may be necessary, even after a successful Kasai procedure. Children with biliary atresia and significant liver damage may need a liver transplant by about age 2.
Children and young adults with biliary atresia can still experience a good quality of life. With proper care and medical oversight, patients can effectively manage their symptoms.
Approximately 80 percent of patients will need a liver transplant by age 20. Even with successful Kasai surgery, most patients will eventually need a transplant because gradual organ damage continues. The timing of the transplant depends on the health of the liver. Many patients can live with their native liver into early adulthood before needing a transplant. Children who have repeated infections (cholangitis) may need a transplant sooner because of damage caused by infection.
Patients with biliary atresia are followed very closely by a gastroenterologist to be sure their nutritional needs are being met and they are growing well. This is a lifelong concern for patients.
Biliary atresia interferes with digestion and food absorption, so patients may suffer from vitamin and protein deficiencies. Because liver disease prevents children from absorbing the same amount of calories from food that healthy children absorb, your doctor may recommend a special diet and visits with a pediatric dietitian.
If your newborn is experiencing symptoms of biliary atresia, like prolonged jaundice or light-colored stools, it is important to speak with the pediatrician as soon as possible. When biliary atresia is diagnosed early, patients are more likely to be candidates for surgery and may be able to delay a liver transplant.
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Pediatric General & Thoracic Surgery
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Houston, Texas 77030
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