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Key Features

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Essentials of Diagnosis
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  • Splenomegaly

  • Recurrent ascites

  • Variceal hemorrhage

  • Hypersplenism

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General Considerations
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  • Defined as an increase in the portal venous pressure to more than 5 mm Hg greater than the inferior vena caval pressure

  • Most commonly a result of cirrhosis

  • Causes of portal hypertension without cirrhosis may be divided into

    • Prehepatic

    • Suprahepatic

    • Intrahepatic

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Prehepatic portal hypertension
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  • A history of neonatal omphalitis, sepsis, dehydration, or umbilical vein catheterization may be present

  • Causes in older children include local trauma, peritonitis (pyelophlebitis), hypercoagulable states, and pancreatitis

  • A variety of portal or splenic vein malformations, some of which may be congenital, have been described, including defects in valves and atretic segments

  • Cavernous transformation is probably the result of attempted collateralization around the thrombosed portal vein rather than a congenital malformation

  • The site of the venous obstruction may be anywhere from the hilum of the liver to the hilum of the spleen

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Suprahepatic portal hypertension (Budd-Chiari Syndrome)
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  • No cause can be demonstrated in most instances in children

  • The occasional association of hepatic vein thrombosis in inflammatory bowel disease favors the presence of endogenous toxins traversing the liver

  • Vasculitis leading to endophlebitis of the hepatic veins has been described

  • Congenital vena caval bands, webs, a membrane, or stricture above the hepatic veins are sometimes causative

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Intrahepatic portal hypertension
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  • Veno-occlusive disease (acute stage)

    • Occurs most frequently in bone marrow or stem cell transplant recipients

      • Develops in the first month after bone marrow transplantation

      • Characterized by the triad of weight gain (ascites), tender hepatomegaly, and jaundice

    • Additional causes include

      • High-dose thiopurines

      • Ingestion of pyrrolizidine alkaloids ("bush tea") or other herbal teas

      • Familial form of the disease occurring in congenital immunodeficiency states

  • Congenital hepatic fibrosis

    • Rare autosomal recessive cause of intrahepatic presinusoidal portal hypertension

    • Autosomal recessive polycystic kidney disease is frequently associated with this disorder

  • Other rare causes

    • Hepatoportal sclerosis (idiopathic portal hypertension, noncirrhotic portal fibrosis)

    • Focal nodular regeneration of the liver

    • Schistosomal hepatic fibrosis

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Clinical Findings

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Symptoms and Signs
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Prehepatic portal hypertension
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  • Symptoms may occur before age 1 year, but in most cases the diagnosis is not made until age 3–5 years

  • Splenomegaly in an otherwise well child is the most constant physical sign

  • Ascites may be noted

  • Hematemesis and melena are presenting symptoms

  • Diagnosis is suggested by

    • An episode of severe infection in the newborn period or early infancy—especially omphalitis, sepsis, gastroenteritis, severe dehydration, or prolonged or difficult umbilical vein catheterizations

    • No previous evidence of liver disease

    • A history of well-being prior to onset or recognition of symptoms

    • Normal liver size and tests with splenomegaly

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Suprahepatic portal hypertension
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  • Most patients have abdominal pain, tender hepatomegaly of acute onset, and ascites

  • Jaundice is present in only ...

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