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.
This condition is usually managed nonoperatively.
Surgery should be performed within 60 days of life.
Cystoenterostomy provides adequate biliary drainage.
Most of these patients will not require transplantation.
Pediatric Surgery