Severe autosomal dominant polycystic kidney disease

Description 

A 61-year-old man with a known history of autosomal dominant polycystic kidney disease (ADPKD) and stage IV chronic kidney disease presented with a 6-month history of abdominal pain, nausea, vomiting and fatigue. In addition to the ADPKD, the right kidney had a 4.4 cm inferior pole mass concerning for renal cell carcinoma (RCC). Preoperative imaging (figure 1), a coronal CT of abdomen and pelvis, demonstrates bilateral ADPKD. His total kidney volume was calculated to be 9980.5 mL, which in combination with his age made him a '1E' (most severe) based on the Mayo Clinic risk stratification schema. The following aggregate of issues led the patient to undergo bilateral open nephrectomies: the suspicion for malignancy associated with the right renal mass, a slight increase in malignancy risk associated with polycystic kidney disease in general, and the likely need for postoperative dialysis if left with only a single poorly...

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