Definition:

Biliary atresia (BA) is a congenital, fibro-obliterative obstructive cholangiopathy. It can be a devastating disease in infants invariably leading, if untreated, to cirrhosis, liver failure and death. Patients will be jaundiced and will have pale stools and dark urine. This is due to the inability to excrete conjugated bilirubin into the gastrointestinal tract, which is then excreted into the urine causing its color to darken. The cause of BA is not known with any degree of certainty, though there are a number of hypotheses. One prominent theory regarding the pathogenesis is that bile duct injury is initially caused by a viral infection, and then perpetuated by an autoimmune disorder. Surgical drainage, the only effective intervention, is successful only half of the time. Approximately 80% of patients require one or more liver transplantations with the associated morbidity and mortality issues caused by life-long transplant-related immunosuppression.