Arteriohepatic Dysplasia. II. Hepatobiliary Morphology
Ellen I. Kahn, Fredric Daum, James Markowitz, Harvey W. Aiges, Keith M. Schneider, Henry B. So, Peter Altman, Roma S. Chandra, Mervin Silverberg – 1 January 1983 – Five children were noted to have arteriohepatic dysplasia (Alagille's syndrome) between 3 and 7 months of age. Prior to diagnosis, four underwent Kasai procedures after intraoperative cholangio‐grams failed to demonstrate patency of the extrahepatic bile ducts. In three patients, a focal proximal hypoplasia of the common hepatic duct was demonstrated with fibrosis and increased vascularity.