Arteriohepatic Dysplasia. II. Hepatobiliary Morphology

Authors

  • Ellen I. Kahn,

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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  • Fredric Daum,

    Corresponding author
    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
    • Fredric Daum, M.D., Chief, Division of Pediatric Gastroenterology, North Shore University Hospital, 300 Community Drive, Manhasset New York 11030.
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  • James Markowitz,

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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  • Harvey W. Aiges,

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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  • Keith M. Schneider,

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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    • Deceased

  • Henry B. So,

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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  • Peter Altman,

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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  • Roma S. Chandra,

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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  • Mervin Silverberg

    1. Department of Laboratories, Pediatrics and Surgery, North Shore University Hospital, Manhasset, New York 11030; Cornell University Medical College, New York, New York 10021; Department of Surgery, Babies Hospital, Columbia Presbyterian Medical Center, New York, New York 10032; Department of Anatomic Pathology, Children's Hospital National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010
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  • This work was presented in part at the International Conference on Biliary Atresia on November 14, 1981 at the North Shore University Hospital, Manhasset, New York.

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.

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