The remaining 15 percent have some degree of liver disease. Older children may continue to have good bile drainage and no jaundice.Įighty-five percent of all children who have biliary atresia will need to have a liver transplant before they are 20 years old. Nearly half of all infants who have had a Kasai procedure require liver transplantation before age 5. If this is the case, liver transplantation can correct this problem. In 15-40 percent of patients the Kasai procedure does not work. The Kasai procedure is not a cure for biliary atresia, but it does allow babies to grow and have fairly good health for several, sometimes for many, years. This means that bile drains from the liver and the jaundice level goes down. The surgeon then attaches a loop of intestine to this portion of the liver, so that bile can flow directly from the remaining healthy bile ducts into the intestine. With an experienced surgeon, the Kasai procedure is successful in 60 to 85 percent of the patients. The surgeon removes the damaged ducts outside of the liver (extrahepatic ducts) and identifies smaller ducts that are still open and draining bile. It is named after the surgeon who developed it. The Kasai procedure is an operation to re-establish bile flow from the liver into the intestine. A Kasai procedure or hepatoportoenterostomy is done. Diagnostic surgery with operative cholangiogram confirms diagnosis.īiliary atresia cannot be treated with medication. Biliary atresia is diagnosed through blood tests, xrays to look for an enlarged liver, and liver biopsy. Symptoms of the disease typically appear within the first two weeks to two months of life. Symptoms include: jaundice, dark urine, clay-colored stools, weight loss or irritability. For other children with biliary atresia, the bile ducts may be damaged by the body's immune system in response to a viral infection acquired after birth.īabies with biliary atresia usually appear healthy when they are born. For some children, biliary atresia may occur because the bile ducts did not form properly during pregnancy. The causes of biliary atresia are not completely understood. This causes the bile to be trapped inside the liver, quickly causing damage and scarring of the liver cells (cirrhosis), and eventually liver failure. When a baby has biliary atresia, bile flow from the liver to the gallbladder is blocked.
When the biliary system is working the way it should, it lets the bile drain from the liver into the intestines. This network of channels and ducts is called the biliary system. It also carries waste products from the liver to the intestines for excretion. Cells within the liver produce liquid called bile. Symptoms of the disease appear or develop about two to eight weeks after birth. Fortunately, despite recurrent pain, these children have normal growth and are generally healthy.īiliary atresia is a rare disease of the liver and bile ducts that occurs in infants. These children often miss school and activities. The pain can be triggered by illness, stress, constipation, or other factors. In some children, the nerves become very sensitive, and pain is experienced even during normal intestinal functions. A child’s intestine has a complicated system of nerves and muscles that helps move food forward and carry out digestion.
Nevertheless, the pain is very real, and is due to extra sensitivity of the digestive organs, sometimes combined with changes in gastrointestinal movement patterns. Your GI Provider will help determine whether your child’s pain is functional. The term “functional” refers to the fact that there is no blockage, inflammation or infection causing the discomfort. Most otherwise-healthy children who repeatedly complain of stomachaches for two months or more have functional abdominal pain.