Intrahepatic portal venous hypoplasia in Alagille's syndrome

One new case of arteriohepatic dysplasia (Alagille's syndrome) is reported. The case showed signs of progressive portal hypertension with the liver biopsy revealing evidence of hypoplasia of the intrahepatic portal venous branches without evident cirrhosis, next to the characteristic paucity of intrahepatic bile ducts. This venous abnormality might be responsible for the unfavourable outcome observed in some cases with Alagille's syndrome and corresponds to a severe form of the disease.