Variation in liver histology in Alagille's syndrome

A previously undescribed variation in liver histology was observed in association with syndromatic neonatal jaundice, otherwise known as Alagille's syndrome. While hepatic ductular hypoplasia is thought to be characteristic of this disorder, we observed a patient who had all of the characteristic morphological features of Alagille's syndrome but whose liver biopsy demonstrated marked ductular proliferation more characteristic of biliary atresia. This histological feature has not been previously reported with Alagille's syndrome and hepatic ductular hypoplasia can no longer be considered characteristic of syndromatic neonatal jaundice.