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

Korean Journal of Nephrology 2007;26(6):772-778.

Tae-Woo Kim, M.D.1, Sook Kim, M.D.2 , Seung Boo Yang, M.D.3 Chu Hee Lee, Ph.D.4, Jun Young Do, M.D.5, Jong Won Park, M.D.5

Department of Internal Medicine¹
Pathology2 and Radiology³
Soonchunhyang Hospital Gumi, Gumi, Korea Department of Biochemistry and Molecular Biology4 and Internal Medicine5 College of Medicine, Yeungnam University, Daegu, Korea

증례 : 다낭성 신질환을 동반한 만성신부전으로 혈액투석 중인 결절성 경화증 환자에 발생한 신혈관근지방종 파열 치료 1예

김태우1 김 숙2 양승부3 이추희4 박종원5 도준영5

순천향대학교 구미병원 내과1, 병리과2, 영상의학과3, 영남대학교 의과대학 생화학분자생물학교실4, 내과학교실5

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.

Key Words: Tuberous sclerosis, Autosomal dominant polycystic kidney, Angiomyolipoma