The Alagille syndrome (arteriohepatic dysplasia).

Alagille et all 2 first drew attention to a group of children with hypoplastic intrahepatic bile ducts but patent extrahepatic ducts. Watson and Miller3 and Alagille et aP reported two series of children with chronic cholestasis due to this cause, noting con- genital heart disease, a characteristic facies, and minor skeletal abnormalities as associated findings with what was felt to be a distinct recognisable syndrome, which is known as the Alagille syndrome or arteriohepatic dysplasia. Subsequent case reports have drawn attention to the ocular involvement seen in persons with this disorder and its familial nature." I