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Arteriohepatic Dysplasia. II. Hepatobiliary Morphology
Author(s) -
Kahn Ellen I.,
Daum Fredric,
Markowitz James,
Aiges Harvey W.,
Schneider Keith M.,
So Henry B.,
Altman Peter,
Chandra Roma S.,
Silverberg Mervin
Publication year - 2007
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840030113
Subject(s) - cholestasis , biliary atresia , medicine , alagille syndrome , porta hepatis , dysplasia , hypoplasia , bile duct , gastroenterology , gallbladder , atresia , fibrosis , congenital hepatic fibrosis , vascularity , pathology , portal hypertension , liver transplantation , cirrhosis , transplantation
Abstract 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. Hypoplasia of the gallbladder occurred in two patients. Changes were observed in the porta hepatis. Eighty to 208 /tm bile ducts were associated with peripherally located gland‐like structures. These changes are indistinguishable from those in fibrous remnants of extrahepatic biliary atresia. Hepatic features of sequential liver biopsies obtained in the five patients were divided into early and late changes. From birth to 3 months of age, the pathology consisted of cholestasis and bile duct destruction. After 3 months of age, there was persistent cholestasis, paucity of interlobular bile ducts, and portal fibrosis. Ductular proliferation was not an intrinsic change. When present, it was related to a recent episode of cholangitis.