Diseases & Conditions


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Biliary Atresia


Every year around 50 babies a year are born in England and Wales with biliary atresia. Once the condition is spotted, they are sent for an operation called Kasai-poroenterostomy. If this fails, they will require a liver transplant. Three centres in England have expertise in the operation. What is biliary atresia? Biliary atresia is caused when the bile duct to the liver becomes inflamed. This is normally due to damage caused around the time of birth. It is not clear how or why this occurs. The bile duct becomes blocked and means bile builds up in the liver and begins to poison it. The child will develop jaundice. Other symptoms include yellow-coloured urine and pale stools. Many children suffer jaundice in infancy, but concerns are usually linked to children who have the condition for longer than two weeks. Doctors warn that the earlier it is caught, the less damage it will have done to the liver. Operations to cure the condition are most successful in children under eight weeks old. How is the condition diagnosed? A battery of tests are often required to confirm a diagnosis of biliary atresia, as the symptoms can be confused with other conditions. These can include: A blood test to look for raised levels of bilirubin An abdominal x-ray to look for an enlarged liver and spleen Abdominal ultrasound to examine the liver and bile ducts An HIDA scan to determine how well bile is flowing A liver biopsy to determine degree of cirrhosis or to rule out other causes of jaundice If the test results suggest biliary atresia, an operation is performed to examine the bile ducts and an x-ray is taken during the operation to determine whether the bile duct is blocked. How is biliary atresia treated? The condition is treated through an operation called Kasai-portoenterostomy. This involves using a loop of bowel to form a duct to drain the bile from the liver. The operation is named after the Japanese surgeon, Professor Morio Kasai, who developed it in 1959. It was first introduced in the UK in the 1960s. The operation is extremely intricate and its success depends on the skill of the surgeon, whether he or she carries out the operations fairly regularly and the support available to the child before and after surgery. Operations can be carried out on children as young as 11 days old. If surgery fails, the child's only other option is a liver transplant, which is a traumatic operation with varying degrees of success. According to the Children's Liver Disease Foundation, the operation costs between £5,000 and £7,000 and the child may also have to take a course of drugs. A liver transplant costs £40,000 plus the cost of anti-rejection drugs. There are three centres which the CLDF considers expert in performing Kasai operations: Birmingham Children's Hospital, King's College Hospital in London and St James' Hospital in Leeds. The first two were named as supra-regional liver units by the government in 1985, but this designation has since been taken away because of changes in the way the NHS is run. St James' was included because paediatric surgeon Mark Stringer trained under an expert in the field and his rate of success is similar to King's College's. This page contains basic information about biliary atresia. For more information, consult your doctor.