Biliary atresia is a paediatric condition involving either obliteration or discontinuity within the extrahepatic biliary system, which results in an obstruction in the flow of bile
Aetiology
More common in females than males
Biliary atresia is unique to neonatal children: the perinatal form presents in the first two weeks of life, and the postnatal form presents within the first 2-8 weeks of life
Pathophysiology
The pathogenesis of biliary atresia is unclear, however, infectious agents, congenital malformations and retained toxins within the bile are all contributing factors
Types
Type 1: the proximal ducts are patent, however, the common duct is obliterated
Type 2: there is atresia of the cystic duct and cystic structures are found in the porta hepatis
Type 3: there is atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia
Clinical presentation
Symptoms
Patients typically present in the first few weeks of life with:
Jaundice extending beyond the physiological two weeks
Dark urine and pale stools
Appetite and growth disturbance, however, may be normal in some cases