Managing a massive renal angiomyolipoma

A giant renal angiomyolipoma (AML) measuring 18 × 13.2 × 22.4 cm was found in the left kidney of this 43-year-old female (with no evidence of tuberous sclerosis [TS]). Her other kidney was atrophic contributing only 3% of total renal function (damaged by chronic pelvi-ureteric junction obstruction). The grossly hypertrophied left kidney was causing displacement of the ureter, pancreas and spleen (Figure 1). There was gross neovascularization within the AML, and the patient suffered three life threatening episodes of haemorrhage requiring interventional radiological procedures which attempted to coil the arteriovenous malformations. On one of these occasions the procedure caused a renal artery aneurysm and a radiological attempt at repair resulted in contrast induced anaphylaxis thereby precluding any further radiological attempts. Owing to the risk of further haemorrhagic episodes, a difficult decision was made to attempt a partial nephrectomy to excise the angiomyolipoma – at this risk of rendering the patient effectively anephric and dialysis dependent if total nephrectomy had to be performed to control bleeding. Intraoperatively the surgeons were able to successfully resect a segment of AML measuring 14 × 19 cm. Postoperatively there was a prolonged period of hypotension requiring inotropic support and blood product replacement. She underwent three further abdominal operations to investigate and treat ongoing blood loss and abdominal compartment syndrome. This was subsequently exacerbated by intra-abdominal sepis – perihepatic collections necessitating ultrasound guided drainage and episodes of pneumoniae with multiple courses of intravenous antibiotics. During her ITU course she required haemofiltration, ventilation via a tracheostomy and total parenteral nutrition. Upon clinical improvement a right sided homonymous hemianopia was noted, with evidence of a left posterior circulation artery territory ischaemia demonstrated on head MRI scan (Figure 2). The patient made a very slow recovery and was discharged 50 days later, with normal renal function, a large open abdominal wound (left to heal by secondary intention) and a new visual field defect. She has subsequently developed an incisional hernia and the visual defect has failed to resolve.