Biliary atresia is a rare congenital disorder in which bile ducts in the liver become blocked or do not develop correctly. When the ducts do not function properly, the liver is unable to transfer bile and salts to the small intestine, leading to inflammation and scarring of liver tissue. Newborns usually begin to show symptoms of biliary atresia within three weeks, which include jaundice, weight loss, irritability, and fatigue. Babies who receive immediate treatment are often able to recover with little or no permanent damage to their internal structures. Without treatment, however, infants are subject to cirrhosis, infections, liver failure, and potentially death.
Healthy bile ducts carry the fluid produced in the liver to the gallbladder and small intestine. Bile is helpful in breaking down fats, disposing waste products, and introducing nutrients into the body. Biliary atresia occurs when ducts inside or around the liver are unable to transfer bile, causing a buildup of fluid in the liver. Excess bile in the liver quickly leads to cell damage and scarring of delicate liver tissue.
Babies with biliary atresia usually appear normal at birth, but start to show signs of liver problems after two or three weeks. They usually suffer from jaundice, where the eyes and skin turn yellow, stools become lighter in color, and urine darkens. Jaundice and digestive problems can lead to irritability, weight loss, and a lack of activity.
A physician who suspects that a newborn may have biliary atresia can perform a number of clinical tests. The doctor usually begins by conducting a physical examination, where he or she presses on the baby's abdomen to check for swelling or an irregular sized liver. Experts may administer x-rays and ultrasounds, order blood tests, or extract liver tissue for a biopsy. Physical exams and laboratory tests allow medical professionals to rule out other liver maladies and make a proper diagnosis of biliary atresia.
Once doctors have determined that an infant has biliary atresia, surgery is almost always required to relieve symptoms and promote normal development. When the condition is discovered within two months of birth, a surgeon may attempt a Kalai procedure, which involves removing the damaged bile duct and attaching the liver directly to the small intestine. There is a significant risk of infection during or after a Kalai procedure, and physicians usually prescribe mild antibiotics to combat bacteria. The best option for infants over two months old is a liver transplant, wherein the surgeon completely removes the liver and bile ducts and replaces them with healthy donor organs. Most infants who receive treatment within their first four months of life are likely to recover completely.