Portal hypertension is rare in children in the United States, but is one of the common causes of major upper GI hemorrhage. Extrahepatic portal thrombosis, parenchymal liver disease associated with fibrocystic disease, and biliary cirrhosis in youngsters with congenital biliary atresia surviving as a result of portal enterostomy are examples of conditions that can result in portal hypertension and esophagogastric varices. In two-thirds of cases, no specific cause is found.
Massive hematemesis is the usual initial manifestation, along with hematochezia in children, whereas ascites is more common as the presenting sign in infants. Usually, the bleeding is self-limited. A nasogastric tube can be placed to empty the stomach and to monitor for continued bleeding and blood transfusions given as indicated. Correct any coagulation abnormalities. Emergency consultation with a surgeon, pediatric surgeon, or a pediatric gastroenterologist is necessary.
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