pediatrics
Biliary Atresia
Treatment of Biliary Atresia
Intraoperative cholangiogram Portojejunostomy (Kasai procedure) Frequently liver transplantation Infants with presumed biliary atresia require surgical exploration with an intraoperative cholangiogram. If biliary atresia is confirmed, a portojejunostomy (Kasai procedure) should be done as part of the same surgical procedure. Ideally, this procedure should be done in the first month of life. If done 60 days or more after birth, the prognosis significantly worsens. ) are frequently prescribed for a year postoperatively in an attempt to prevent ascending cholangitis. Medications that increase bile production (choleretic agents), such as ursodiol, are frequently used postoperatively. Nutritional therapy including supplemental fat-soluble vitamins (A, D, E, and K) is very important to ensure adequate intake to support growth ( Treatment reference ). Even with optimal therapy, approximately 50% of infants will develop cirrhosis and require liver transplantation. Frequently, infants who cannot undergo portojejunostomy require liver transplantation by 1 year of age. 1.Mouzaki M, Bronsky J, Gupte G, et al: Nutrition support of children with chronic liver diseases: A joint position paper of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition.J Pediatr Gastroenterol Nutr69(4):498–511, 2019. doi: 10.1097/MPG.0000000000002443 