Budd-Chiari syndrome in autosomal dominant polycystic kidney disease: a complication of nephrectomy in patients with liver cysts.

We report two patients with autosomal dominant polycystic kidney disease (ADPKD) who developed the Budd-Chiari syndrome following bilateral nephrectomy. Both patients had massive cystic enlargement of the liver. Neither had any other identifiable risk factors for the Budd-Chiari syndrome. We suggest that removal of the kidneys may predispose toward anatomic obstruction of the inferior vena cava or hepatic veins by liver cysts. Nephrectomy should be approached cautiously in ADPKD patients with extensive involvement of the liver by cysts.