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Hepatoportal sclerosis in childhood

J G Maksoud, S Mies, L C da Costa Gayotto

    American Journal of Surgery
    |April 1, 1986
    PubMed

    Insights

    Hepatosplenomegaly in children may indicate hepatoportal sclerosis, characterized by intrahepatic portal vein obstruction. This condition involves venous changes and can be associated with extrahepatic obstructions, impacting liver function.

    Area of Science:

    • Pediatric Gastroenterology
    • Vascular Pathology
    • Hepatology

    Background:

    • Hepatosplenomegaly with preserved liver function and esophageal varices are key clinical signs.
    • Histological findings reveal prominent portal alterations, including subintimal sclerosis of intrahepatic venous branches and telangiectases.

    Purpose of the Study:

    • To present the clinical, angiographic, and pathologic features of hepatoportal sclerosis in children.
    • To investigate the primary cause and extent of portal vein obstruction in pediatric hepatoportal sclerosis.

    Main Methods:

    • Clinical case review of five pediatric patients.
    • Angiographic assessment of portal venous system.
    • Pathologic examination of liver tissue.

    Main Results:

    • All patients demonstrated intrahepatic portal venous obstruction.
    • Three patients also had associated extrahepatic portal vein obstruction.
    • Consistent and prominent portal alterations were observed histologically.

    Conclusions:

    • Intrahepatic portal vein thrombosis is suggested as the primary disorder in hepatoportal sclerosis.
    • The extent and site of venous involvement can vary, affecting intrahepatic branches and potentially the portal trunk.
    • Integrating clinical, angiographic, and pathologic data enhances understanding of pediatric hepatoportal sclerosis.

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