Portal obstruction in children. I. Clinical investigation and hemorrhage risk.

We examined 108 children with obstruction of the portal vein. Symptoms included splenomegaly and gastrointestinal tract hemorrhage. Obstruction was secondary to portal vein injury in 44 children and was combined with congenital malformations in 17 others. Ultrasonography provided the correct diagnosis in 36 of the 37 children in whom it was performed. Angiography, performed in 101 children, showed that the obstruction extended to the superior mesenteric vein in 14 children and to the entire portal venous system in seven; intrahepatic branches were involved in half the cases. Natural splenorenal shunts were visible in 19 children but were not clearly associated with a lower risk of gastrointestinal tract bleeding; in five of 30 children, cavography displayed abnormalities of the inferior vena cava. Spontaneous gastrointestinal tract hemorrhage occurred in 78 children. Fiberoptic endoscopy showed esophageal varices in 79 of the 81 children studied. The presence of tense varices and congestion of esophageal mucosa clearly augmented the risk of bleeding. These results suggest a simple method of investigation based on ultrasonography for diagnosis and on endoscopy for prognosis. Angiography should be limited to children with a history of gastrointestinal tract bleeding for whom a surgical portosystemic shunt is being considered.