Why does biliary atresia cause jaundice

But in babies with biliary atresia, it lasts longer because the bile ducts begin closing up. Other liver conditions that cause liver inflammation like infections and genetic conditions cause the same symptoms as biliary atresia. So doctors do tests to diagnose the problem, such as:.

Biliary atresia needs quick treatment by liver specialists and surgeons. Babies with blocked bile ducts need a surgery called the Kasai procedure. This lets bile drain from the liver directly into the small intestine. At the start of the surgery, the doctor will do a cholangiogram to see if bile ducts are blocked. Most babies with biliary atresia will need a liver transplant later in childhood or before they become adults. The specific diagnosis of biliary atresia requires blood and x-ray tests, and sometimes a liver biopsy.

If biliary atresia is suspected, the newborn will need to be evaluated by a pediatric liver specialist such as a pediatric hepatologist liver disease in children , pediatric gastroenterologist, digestive disease in children or pediatric surgeon with expertise in surgery of the liver and bile ducts.

Biliary atresia can often be difficult to diagnose. A combination of diagnostic tests will be used to including:. If biliary atresia appears to be the cause of jaundice, surgery to examine the bile ducts is performed.

If the surgeon confirms the diagnosis of biliary atresia, a Kasai Procedure hepato-portoenterostomy is performed to reconstruct the bile ducts and restore bile flow. In the Kasai Procedure, the blocked extrahepatic bile ducts as well as the gallbladder and replaced by a segment of the child's intestine, which is sewn directly to the liver.

The surgical joining of the liver with a segment of the intestine functions as the new extrahepatic bile duct system. If the Kasai procedure is unsuccessful, the infant will usually require a liver transplant, sometimes between ages 1 and 2. Of all infants who have had a Kasai procedure, fully half still require liver transplantation before age 5.

Clinical trials are underway to improve the outcome of the Kasai or gall-bladder Kasai in infants with biliary atresia. Children with the fetal form of biliary atresia are more likely to require liver transplants, usually sooner rather than later, than for infants with the more usual perinatal form of the disease. The need for a transplant is also affected by the arrangement of diseased bile ducts and the extent of damage.

Liver transplantation is a highly successful treatment for biliary atresia. Survival after surgery has increased dramatically in recent years. Children with biliary atresia are now surviving well into adulthood. Improvements in transplant surgery have also led to a greater availability of livers for transplantation in children.

In the past, only livers from small children were deemed a suitable match for a transplant. Today, however, advances in treatment now allow the option of "reduced size" or "split liver" transplants, in which a piece of an adult liver can be used for transplantation in a child with biliary atresia. Parents or relatives of children with biliary atresia may now be considered potential donors.

This causes liver damage. A liver transplant may also be needed over time. A liver transplant removes the damaged liver and replaces it with a new liver from a donor.

The new liver can be one of the following:. After a liver transplant, the new liver begins working and the child's health often gets better quickly. These medicines are called antirejection medicines.

Before your child has surgery, nutrition may be a problem. With biliary atresia, not enough bile reaches the intestine to help digest fats in the diet. Liver damage can cause a lack of protein and a lack of vitamins. Children with liver disease need more calories than a normal child because they have a faster metabolism.

The nutritionist can advise you on your child's diet. Some children with liver disease become too sick to eat normally. The remaining 15 percent have some degree of liver disease. Their disease can be managed without having a transplant. If there is still not enough bile flow with the Kasai procedure, ultimately, liver transplantation will be considered.

A liver transplant operation removes the damaged liver and replaces it with a new liver from a donor. After a transplant, ongoing lifelong care is required.

Frequent contact with physicians and other members of the transplant team is also necessary. All rights reserved. Health Library. What Causes Biliary Atresia? Who Is at Risk for Biliary Atresia? There does not appear to be any link to medications taken during pregnancy. Ten to 15 percent of infants with biliary atresia may be born with other problems in the: Heart Spleen polysplenia Blood vessels inferior vena caval anomalies, preduodenal portal vein Intestine situs inversus or malrotation What Are the Symptoms of Biliary Atresia?

Jaundice caused by an immature liver is common in newborns. It usually goes away within the first week to 10 days of life. A baby with biliary atresia usually appears normal at birth, but develops jaundice at two or three weeks after birth. The bilirubin is then filtered by the kidney and removed in the urine. Bile gives stool its green or brown color, and without such, stool is without color often white or gray.

How Is Biliary Atresia Diagnosed? Blood tests are done to tell if there are liver function abnormalities. They may also identify the cause etiology of jaundice. X-rays of the abdomen look for an enlarged liver and spleen. An abdominal ultrasound can tell if there is a small gall bladder or none at all. The gall bladder is the organ that stores bile. If this organ is missing or absent since birth, that often indicates biliary atresia.

A liver biopsy tells if an infant is likely to have biliary atresia. In a liver biopsy, a tiny sample of the liver is removed with a needle. That sample is then looked at under a microscope. A liver biopsy is very reliable. If the biopsy shows that the infant probably has biliary atresia, further surgery will confirm the diagnosis and treat the condition.