Tuberous Sclerosis Manifested by Spontaneous Rupture of Renal Angiomyolipoma in a Patient with Polycystic Kidney Disease / 대한신장학회지

ABSTRACT

Tuberous sclerosis is an autosomal dominant disorder characterized by seizure, mental retardation and harmatomatous lesions in multiple organs. The renal lesions of tuberous sclerosis are multiple angiomyolipomas often associated with cysts of various sizes. A 47-year-old man who had been on hemodialysis for 12 years was admitted to our hospital because of sudden onset of right flank pain. He had polycystic kidney disease and adenoma sebaceum. Abdominal computed tomography showed an enlarged right kidney with massive hemorrhage, and renal arteriography showed massive bleeding. Immediate transarterial embolization and radical nephrectomy on the right kidney was done. Pathologic examination revealed ruptured renal angiomyolipoma, confirming that he had contiguous gene syndrome. We experienced a case of tuberous sclerosis with spontaneous rupture of renal angiomyolipoma in a hemodialysis patient with autosomal dominant polycystic kidney disease.