Portal Hypertension

Key Features

Essentials of Diagnosis

• Splenomegaly

• Recurrent ascites

• Variceal hemorrhage

• Hypersplenism

General Considerations

• 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

Prehepatic portal hypertension

• 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

Suprahepatic portal hypertension (Budd-Chiari Syndrome)

• 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

Intrahepatic portal hypertension

• 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

Clinical Findings

Symptoms and Signs

Prehepatic portal hypertension

• 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

Suprahepatic portal hypertension

• Most patients have abdominal pain, tender hepatomegaly ...