Extrahepatic portal venous obstruction. A retrospective analysis.

Extrahepatic portal venous obstruction is a frequent cause of portal hypertension in children. In the majority it presents as gastro-intestinal haemorrhage or as incidental splenomegaly on routine medical examination. The diagnosis is suggested by the presence of oesophageal varices in a patient whose liver function is normal and can be confirmed by portal venography. A conservative approach with early intensive management of haemorrhage is advised. Surgical shunts do not reduce the frequency of haemorrhage in most patients. Splenectomy should be avoided in all instances.