An infant girl is found to have persistent jaundice after bih. A metabolic screen is normal, ultrasound demonstrates an absent gallbladder, and a technetium-99m iminodiacetic acid scan shows radionuclide that is concentrated in the liver but not excreted into the intestine. Which of the following is true?
This patient has biliary atresia, a rare disease characterized by fibro-proliferative obliteration of the biliary tree, which progresses toward hepatic fibrosis, cirrhosis, and end-stage liver failure. Surgical treatment is the first-line therapy, consisting of creation of a hepatopooenterostomy (Kasai procedure). Infants treated prior to 60 days of life are more likely to achieve successful biliary drainage than older infants.