A surgical approach to Wilms' tumour with retrohepatic vena caval extension

Wilms' tumours (WT) with retrohepatic vascular extension traditionally requires cardiac bypass for complete excision. We share our experience of these complex cases.

A retrospective review was performed of children with WT with retrohepatic vascular extension presenting to two UK children's hospitals. Tumour stage, chemotherapy, level of vascular extension, operative details and complication data were analysed.

Ten children were identified. Mean age 6.6 years (range 3.3-8.2 years); tumour side 6 right, 2 left, 2 bilateral. Level of tumour extension was to the right atrium in two, diaphragm in two, hepatic vein (HV) level in four and retrohepatic inferior vena cava (IVC) in one patient. Following chemotherapy it reduced to hepatic veins (5) or below (4). Surgery involved radical nephrectomy and complete mobilisation of the liver off the IVC, which was then clamped, opened and the thrombus excised. There were no intraoperative complications. Mean hospital stay was 9.77 days (7-20 days). Histology showed viable tumour thrombus in six patients. One patient died after 1 year from metastatic disease.

Retrohepatic extension of WT can be managed without bypass using pre-operative chemotherapy and by complete liver mobilisation. The tumour was always adherent to IVC and required sharp dissection.