Metastatic Wilms' tumor causing acute hepatic-vein occlusion (Budd-Chiari syndrome).

We describe a case of Wilms' tumor in a 6-yr-old girl which extended from the right kidney and completely obstructed the inferior vena cava and hepatic veins to the level of the right atrium, producing an acute Budd-Chiari syndrome. After an initial course of chemotherapy, the primary tumor was excised and, using cardiopulmonary bypass, the tumor thrombus was removed from the inferior vena cava and hepatic veins. The Budd-Chiari syndrome resolved and after triple-drug chemotherapy and radiotherapy under the National Wilms' Tumor Study-2 protocol the child has remained disease-free for 5 yr.