pediatrics
Biliary Atresia
Introduction
Biliary atresia is obstruction of the biliary tree due to progressive sclerosis of the extrahepatic bile duct. Diagnosis is by blood tests, ultrasonography, liver biopsy, and hepatobiliary scan. Treatment is surgical, with portojejunostomy (Kasai procedure) or liver transplantation. (See also Overview of Congenital Gastrointestinal Anomalies .) The incidence of biliary atresia in the United States is about 1/8,000 to 1/18,000 live births ( General references ). In most cases, biliary atresia manifests several weeks to months after birth, probably after inflammation and scarring of the extrahepatic (and sometimes intrahepatic) bile ducts ( General references ). It is rarely present in premature infants or in neonates at birth (or is unrecognized in neonates). The cause of the inflammatory response is unknown. Several infectious organisms have been implicated, including reovirus type 3 and cytomegalovirus, but no definitive association has been noted. In addition, there may be a genetic component with defects in one of several genes ( Biliary atresia can lead to Cirrhosis , with progressive, irreversible scarring of the liver. Cirrhosis can develop by 2 months of age and progresses if the defect is not treated. Approximately 15 to 25% of infants with biliary atresia have other congenital defects, including polysplenia/asplenia, intestinal atresia, situs inversus, and Overview of Congenital Cardiovascular Anomalies or 