This 27-month-old boy was referred in December 1969 with a very large left-sided Wilms’ tumor. An older sister at age 3½ years had been operated on elsewhere with a large right-sided Wilms’ tumor in 1966. Despite radiation and chemotherapy, she died in 1967, one month after the birth of her younger baby brother.
Intravenous pyelogram of this boy showed nonvisualization of both renal collecting systems. An inferior vena cava study, with a greater volume of contrast, showed obstruction of the cava and extension of the left-sided tumor up the vena cava into the right atrium. Satisfactory visualization of the renal collecting systems on both sides was seen with the increased contrast bolus. Three pulmonary metastases were seen in the right lower lung field. Venous return from both kidneys was via collateral veins rather than the renal veins because of the caval obstruction by tumor. Liver chemistries suggested obstruction of hepatic venous vessels, also producing the Budd-Chiari Syndrome.
Fig. 1. Preoperative Chest Film Showing Diaphragm Pushed Upward by Ascites and Fluid in Right Thorax (Select Image for High-quality Version). Liver palpably enlarged. These findings were secondary to blockage of hepatic venous outflow by tumor in the inferior vena cava. (Budd-Chiari Syndrome)
Fig. 2. Vena Cava Angiograms (Select Image for High-quality Version). (A) Preoperative study showing complete obstruction of intrahepatic I.V.C. by tumor extending from left renal vein up into right atrium. Note extensive para spinal collateral veins. (B) Postoperative study showing open I.V.C. and free flow to right heart after removal of left Wilms’ tumor and extraction of tumor previously blocking I.V.C. Normal I.V.P. right kidney. Liver enlargement, purple color, and congestion disappeared immediately upon extraction of intracaval tumor.
A long transverse abdominal incision was made to give access for removal of the large left Wilms’ tumor, but also entry to the right chest for access to the right atrium where the caval extension of tumor was easily felt through the atrial wall. Tourniquet tapes were passed around the vena below both renal veins of both kidneys. The left renal vein was opened. A smooth common duct forcep was inserted slowly and gently, extracting the plug of tumor back from the atrium, palpating it through the atrial wall simultaneously. It came out intact. When an open ended glass suction device was passed down the cava and up into the atrium there was free bleeding. It was interesting to note that the large, tense, purple liver decongested promptly upon removing the tumor from the vena cava, consistent with Budd-Chiari Syndrome with obstructed hepatic veins. The three metastases were removed from the right lung.
Fig. 3. Surgical Specimen (Select Image for High-quality Version). Tumor hemisected. Capsule had been intact. Regional lymph nodes were negative for tumor. Below the large specimen is the gently extracted 3-inch-long (7.62-cm) plug of tumor, which ended in the right atrium. It appeared to be intact.
Standby cardiopulmonary bypass was available when the plug of tumor was being extracted. Radiation therapy was given to both lungs and the left flank and periaortic region.
An episode of intestinal obstruction occurred two months post-operatively. A perisplenic abscess had eroded into the splenic flexure of the colon. The segment of colon was resected. He convalesced well.
Alternating courses of Actinomycin D and Vincristine were given until February 1973.
In July 1973, when he was age 6 years a complete survey for residual tumor proved negative. His last follow-up at age 7½.
In December 2011, contact was reestablished. Then age 44 years, the once precariously ill lad was married, father of two sons, and employed in strenuous physical work in a warehouse. His health is normal.