A 14-month-old female was referred to our hospital with a history of an enlarging abdominal mass noted by her parents for 3 days. Physical examination revealed an abdominal mass with clear and smooth margins extending from the right upper quadrant to the right hemipelvis. Routine blood tests were normal apart from mild anaemia and urine analysis did not show hematuria. Ultrasonography of the abdomen revealed a unilateral 10.8 × 7.2 × 9.2 cm solid tumor in the right kidney, whereas the contralateral kidney was normal. Computed tomography (CT) revealed a large lesion arising from the inferior aspect of the right kidney, occupying the right flank and extending across the midline. Enhanced CT detected duplication of IVC below the renal veins and compression and displacement of the right IVC caused by the enormous tumor (Fig. ). An additional movie file shows this in more detail [see Additional file ].\nNeither intravascular extension nor invasion to adjacent organs and regional lymph nodes was detected by CT. Chest radiography was reported normal.\nWith the presumptive diagnosis of WT, a right-sided radical nephrectomy was performed. Final pathology was consistent with favorable histology, stromal-predominant (60%) WT. The renal vessel and IVC were tumor free. The renal hilar and para-aortic lymph nodes were also free from tumor and the final pathological stage was Stage I. According to the regimen of the National Wilms’ Tumor Study Group 5, the patient received postoperative chemotherapy with dactinomycin and vincristine. CT imaging at 3 months postoperatively showed no evidence of residual or recurrent disease. Interestingly, the right IVC played a dominant role and the left IVC seemed to disappear in postoperative enhanced CT (Fig. ). An additional movie file shows this in more detail [see Additional file ]. During the follow-up of 18 months, no local recurrence or metastasis has been observed.
A 35-year-old man with the diagnosis of mitral atresia, a double outlet right ventricle, pulmonary atresia with interrupted inferior caval vein, and azygous continuation to the right superior caval vein underwent a Blalock-Taussig shunt in early childhood and a Kawashima operation at the age of 17 at another institution. He presented to our clinic with progressive cyanosis and fatigue 18 years after his last surgical procedure. His oxygen saturation was 65%.\nCardiac catheterization revealed left pulmonary artery stenosis and a decompressing vein from the right superior caval vein to the left superior caval vein which directly drains to the left atrium. Left pulmonary artery stenting was done via right internal jugular approach (Figures , , and ).\nHe underwent a second catheterization for occlusion of the left superior caval vein via left internal jugular approach. The anomalous vein measured 22 and 20 mm in AP and lateral projections, respectively. Balloon test occlusion resulted in immediate increase in oxygen saturation from 65% to 82% without drop of blood pressure. The mean pressure in the azygous vein remained at 9 mmHg; pressure in the left internal jugular vein increased to 11 mmHg and dropped within a few minutes to 10 mmHg. Angiography showed good venous collateral drainage.\nThe largest available vascular plug, 24 mm in diameter, failed to completely occlude the vessel, which was successfully occluded using a 24 mm ASD occluder (Cera Lifetech, China) via a 12 Fr sheath. Upon completion of the procedure the patient had oxygen saturation of 80%, left internal jugular pressure of 10 mmHg, and right superior caval vein and pulmonary artery pressure of 9 mmHg.\nAfter occlusion, the patient had significant improvement of his exercise intolerance and fatigue. Follow-up hemodynamic study 12 months later showed no increase in venous pressure, no development of venous collaterals, and no decrease in his oxygen saturation.