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Vascular Liver Disease

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Book cover Diseases of the Liver in Children

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Abstract

Vascular liver disease may affect the portal vein, the sinusoids, or the hepatic veins, and may be congenital or develop later during childhood. Clinical presentations vary markedly, depending on the location of the disease in the hepatic vascular anatomy, the nature of the pathology, the degree of hemodynamic change, and the acuity of onset of any vascular obstruction. Affected children may develop clinical manifestations of portal hypertension (e.g., ascites, gastrointestinal bleeding), portosystemic shunting (e.g., encephalopathy, hepatopulmonary syndrome), liver failure, liver masses, or high-output heart failure. Accurate diagnosis depends mostly on imaging. Treatments are directed at specific pathologies and hemodynamic abnormalities, and include medical therapies (e.g., nonspecific beta-blockers, defibrotide), interventional radiology procedures, and surgery. We describe the current approach to children with portal vein thrombosis, Budd-Chiari syndrome, sinusoidal obstruction syndrome, idiopathic non-cirrhotic portal hypertension, congenital vascular malformations, and liver vascular tumors.

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Authors and Affiliations

  1. Division of Gastroenterology, Hepatology and Nutrition, Department of Paediatrics, University of Toronto and The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Ines Loverdos MD & Simon C. Ling MBChB, MRCP

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  1. Ines Loverdos MD
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  2. Simon C. Ling MBChB, MRCP
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Correspondence to Simon C. Ling MBChB, MRCP .

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  1. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Karen F. Murray

  2. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Simon Horslen

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Loverdos, I., Ling, S.C. (2014). Vascular Liver Disease. In: Murray, K., Horslen, S. (eds) Diseases of the Liver in Children. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9005-0_21

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