buddchiari

OBJECTIVES: Living-donor liver transplant for BuddChiari syndrome is particularly challenging because of the need for venous outflow reconstruction as grafts from living donors lack vena cava. In addition, recipient vena cava may be thrombotic and fibrotic to such an extent that it would not allow graft venous outflow reconstruction. Under these circumstances, the right atrium provides an easily accessible alternative for venous outflow reconstruction, omitting the need for vena cava replacement...

A 30 year old female with an unexpected right adrenal phaechromacytoma invading the renal vein, the inferior vena cava and extending into the right atrium is presented. She also had BuddChiari syndrome due to invasion of the hepatic veins by the tumour. Additionally, the tumour had metastasised to the liver and the lungs. Despite elevated 24 hour urinary vanillylmandelic acid (VMA) the patient was normotensive pre-operatively. The patient underwent right adrenalectomy and extended nephrectomy with milking of the tumaur from the inferior vena cava...