An infant girl is found to have persistent jaundice after birth. 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?
A. This condition is usually managed non operativelyThis patient has biliary atresia, a rare disease characterized by fibroproliferative 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 hepatoportoenterostomy (Kasai procedure). Numerous studies suggest that the likelihood of surgical success is inversely related to the age at the time of portoenterostomy. Infants treated prior to 60 days of life are more likely to achieve successful and long-term biliary drainage than older infants. Although the outlook is less favorable for patients after the 12th week, it is reasonable to proceed with surgery even beyond this time point, as the alternative is certain liver failure. Approximately one-third of patients remain symptomfree after portoenterostomy; the remainder requires liver transplantation due to progressive liver failure. Independent risk factors that predict failure of the procedure include bridging liver fibrosis at the time of surgery and postoperative cholangitic episodes.