Membranous obstruction of the inferior vena cava with Budd-Chiari syndrome in children: a report of nine cases.

Membranous obstruction of the inferior vena cava (IVC) with Budd-Chiari syndrome is a rare condition in children. Nine children with this lesion are described, eight of them from the Ovambo tribe in Namibia. Clinical features included hepatomegaly, splenomegaly, ascites, and dilated superficial abdominal veins draining cephalad below the umbilicus. Liver enzymes and serum proteins were slightly abnormal in most cases, and the ascitic fluid, when present, had a high protein content. Liver histology showed a wide range of changes, from almost normal to signs of severe chronic congestion. Angiographic appearance varied from a thin membrane between the IVC and right atrium to nonvisualization of the IVC except for dilated collaterals. Simultaneous injection of contrast medium into catheters positioned in the upper IVC and right atrium, through both the femoral and antecubital routes, is the most accurate way of demonstrating the extent of the obstruction and the patency of the hepatic veins.