Biliary Atresia

An obliterative process which involves the common duct, cystic duct, one or both hepatic ducts and the gallbladder. It results in pathologic jaundice (newborn jaundice lasting greater than 2 weeks, with a direct BR of greater than 2 mg/dl). Polysplenia is a commonly associated anomaly, among others. Evaluation involves fasting abdominal ultrasound, needle biopsy of the liver, checking the alpha-1 antitrypsin levels, and technetium 99mIDA scan after pretreatment with phenobarbital. Surgical treatment is the Kasai hepatoportoenterostomy, which is based on the observation that fibrous tissue of the porta hepatis has microscopically patent biliary ducts that communicate with the intrahepatic ductal system. This procedure includes transection of these patent ducts and placing a Roux en y limb of jejunum in communication with them. This can be complicated by postoperative cholangitis. Cure rate with the Kasai procedure is approximately 25-30%. Liver transplantation is performed if the Kasai procedure fails. Biliary atresia is the most common indication for liver transplantation in the pediatric population.

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