A rare condition affecting newborns is biliary atresia, which affects the liver and bile ducts. Bile is a fluid that helps digest fats and remove waste products from the body. Bile ducts carry bile from the liver to the gallbladder and small intestine. In biliary atresia, the bile ducts are either absent or blocked, preventing bile from flowing out of the liver. Bile buildup in the liver can damage the liver cells and cause liver failure.
The exact cause of biliary atresia is unknown, but it is not inherited or contagious. Some possible factors that may contribute to biliary atresia include:
A viral or bacterial infection during pregnancy or after birth that triggers an immune response that damages the bile ducts
A genetic mutation that affects the development of the bile ducts
An abnormality in the embryonic development of the liver and bile ducts
Exposure to toxins or drugs during pregnancy or after birth that affect the liver and bile ducts
The most common symptom of biliary atresia is jaundice, a yellowing of the skin and eyes due to the accumulation of bilirubin, a pigment in bile. Jaundice usually appears within the first two weeks of life and does not improve over time. Other symptoms of biliary atresia include:
Dark urine and pale stools
Poor weight gain and growth
Enlarged liver and spleen
Itching and irritation of the skin
Bleeding and bruising easily
Swelling of the abdomen due to fluid accumulation
Signs of infection such as fever, chills, and vomiting
Biliary atresia can be diagnosed by a combination of tests, such as:
Blood tests to check the levels of bilirubin, liver enzymes, and clotting factors
Urine tests to check for the presence of bilirubin and urobilinogen, a breakdown product of bilirubin
Stool tests to check for the absence of bile
Ultrasound to examine the size and shape of the liver and bile ducts
Liver biopsy to examine a sample of liver tissue under a microscope
Cholangiogram to inject a dye into the bile ducts and take an X-ray to see if they are open or blocked
The main treatment for biliary atresia is surgery to restore the flow of bile from the liver to the small intestine. There are two types of surgery that can be performed:
Usually performed within the first two months of life, the Kasai procedure is the first-line surgery. In order to create a new pathway for bile drainage, the damaged bile ducts are removed and a segment of the small intestine is attached directly to the liver. Although it is not a cure, this surgery can improve biliary atresia symptoms and delay the need for a liver transplant. About 50% to 80% of children who undergo the Kasai procedure will still require a liver transplant later in life, depending on several factors, including the child's age, the extent of liver damage, and the surgeon's skill.
A liver transplant is the definitive treatment for biliary atresia by replacing the diseased liver with a healthy one from a donor. Despite its ability to cure biliary atresia and restore normal liver function, a liver transplant comes with its own risks, including rejection, infection, and bleeding. To prevent the immune system from attacking the new liver, a liver transplant also requires lifelong medication. Approximately 20% to 50% of children with biliary atresia require a liver transplant before the age of 2, and approximately 80% by the age of 20.
There is no known way to prevent biliary atresia, but early diagnosis and treatment can improve the outcome and quality of life for children with this condition. It is therefore important that you seek medical attention if your baby shows signs of jaundice that do not improve or worsen over time, or if any of the other symptoms of biliary atresia occur. It is possible to delay or prevent liver damage and the need for a liver transplant by intervening early.
