Definition 350

Incidence 350

Types 350

Pathogenesis 350

Clinical presentation 351

Diagnosis 351

Treatment 351

Complications 352

Prognosis 352

Biliary atresia (BA) is a progressive cholangiopathy of unknown aetiology affecting extra- and intrahepatic biliary system presenting within the first several weeks of life.

BA is a sporadic condition with an estimated worldwide incidence of around 1/12 000–15 000 live births. It has been described in isolation in twin and triplet pregnancies, with no seasonal pattern. Children are typically born at term, with no gender predominance....