Biliary atresia a liver disease affecting small children. It causes damage, scarring, and blockage of the bile ducts. In healthy babies, bile ducts carry bile from the liver to the gallbladder and eventually to the small intestine. Bile includes chemicals that the body trying to get rid of. When the bile ducts blocked also bile flow stops. This causes these bad chemicals to collect in the liver. This is called cholestasis. Biliary atresia only occurs in young infants (less than 3-4 months of age). Biliary atresia a rare disease also affecting 1 in 8,000 to 1 in 18,000 live births worldwide. About 10-20% of infants with biliary atresia have abnormalities in other organs. such as heart defects or issues with their spleen.
The cause of biliary atresia is unknown. A number of ideas are being tested. One theory suggests viral infection as the possible reason others believe. it may caused by an overactive immune system. A problem in the development of the bile duct, or a chemical or something toxic that damages the bile duct. Genetic reason or a combination of these things also been a postulated cause of biliary atresia. Regardless of the cause and biliary atresia leads to inflammation and scarring of bile ducts outside and inside the liver. The scarring can lead to cirrhosis (severe scarring) by 6 to 12 months of age. When a liver badly scarred it cannot do its job correctly.
DIAGNOSIS OF BILIARY ATRESIA:
Infants who have jaundice that lasts beyond 2-3 weeks of age or who have light-colored stools seen by a doctor. The doctor can check the baby’s blood to see if the liver working correctly. It is very important to diagnose biliary atresia as soon as possible. Because scarring and cirrhosis in the liver can occur rapidly if the disease is not treated. To help make the diagnosis also the medical team may order a number of tests, including.
- Blood and urine tests looking for other common or treatable causes of jaundice.
- Ultrasound to look at the bile ducts, gall bladder, and liver.
- Liver biopsy
- HIDA scan (hepatic scintigraphy) to see if bile is getting into the intestine.
If these tests point to a possible diagnosis of biliary atresia. Your doctor may order the intraoperative cholangiogram. A surgical operation. where the bile ducts searched for and then dye injected directly into the gallbladder to get the best look possible at the bile ducts. If bile ducts cannot seen by this testing. Then the surgical operation is continued to treat the biliary atresia by the Kasai procedure.
TREATMENT OF BILIARY ATRESIA:
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If the testing and cholangiogram show biliary atresia. infants need a surgical operation for treatment also known as the Kasai procedure or hepatoportoenterostomy. In this procedure, the blocked bile ducts removed and a piece of the small intestine is directly connected to the liver in place of the bile duct. This allows bile to leave the liver and enter the intestine. Other supportive medical treatments are recommended before and after the Kasai procedure. These include making sure nutrition ideal by using infant formulas with higher calories or certain types of fat. Doctors might also use additional vitamins, ursodeoxycholic acid, and low-dose antibiotics to prevent bacterial infection of the bile ducts (cholangitis). When the Kasai procedure is successful. The stools become yellow or brown.The bilirubin normalizes in the blood and jaundice disappears, and nutrition and wellbeing improve.
The chance of a successful Kasai procedure best. if the procedure done before 30-45 days of age. Occasionally, infants who not diagnosed until after 3-4 months of age and already have cirrhosis at the time of diagnosis, do not have a Kasai procedure. These infants are then listed for liver transplantation without undergoing a Kasai.
If the Kasai procedure is successful, jaundice disappears and bilirubin levels return to normal. This happens in 40-60% of infants having a Kasai procedure. Even with the Kasai procedure, many children with this disease. still develop more scarring in the liver and eventually need a liver transplant.
Biliary atresia is the most common reason for liver transplantation in children. About half of infants who undergo a Kasai procedure will still need liver transplantation by three years of age. In about one-quarter of infants, jaundice will go away at first. but those children will need liver transplantation by the teenage years because of slowly progressive liver scarring. With our current therapies and only about one-quarter of babies. who the Kasai procedure will survive to their 20’s without needing liver transplantation.
Although liver transplantation a major surgical procedure. Requiring long term immunosuppression and medicines. This life-saving procedure has markedly improved survival rates of infants and children with biliary atresia. Long-term survival after liver transplantation for biliary atresia is over 90%.