Idiopathic Retroperitoneal Fibrosis Associated with a Horseshoe Kidney

Korean Journal of Nephrology 2010;29(3):376-380.

Pyung Kyun Park, M.D.1, Sung Bong Pyo, M.D.1, An Doc Jung, M.D.1, Ji Min Jeong, M.D.1, Dae Hun Lim, M.D.1, Joon-Suk Choi, M.D.1, Hyung Chul Lee, M.D.1, Kyung Jin Oh, M.D.2, Jin Woong Kim, M.D.3 and Nam-Ho Kim, M.D.1

Division of Nephrology¹
Department of Internal Medicine and Urology2 and Radiology³
Chonnam National University Medical School, Gwangju, Korea

증례 : 마제신에 발생한 특발성 후복막 섬유증

박평균1 , 표성봉1 , 정안덕1 , 정지민1 , 임대훈1 , 최준석1 , 이형철1 , 오경진2 , 김진웅3 , 김남호1

전남대학교 의과대학 내과학교실1 , 비뇨기과학교실2 , 영상의학과교실3

Abstract

A case of a 52-year-old man with retroperitoneal fibrosis and a horseshoe kidney is presented. Horseshoe kidney is one of the most common renal anomalies and complicated with urinary tract infection, hydronephrosis, calculi, tumor of the renal pelvis, and other multiple congenital abnormalities. Idiopathic retroperitoneal fibrosis is a rare disease characterized by the presence of a retroperitoneal tissue, consisting of chronic inflammation and marked fibrosis, which often entraps ureters or other abdominal organs. The correlation between horseshoe kidney and retroperitoneal fibrosis has not been described. We report a rare case of idiopathic retroperitoneal fibrosis with horseshoe kidney disease demonstrating good response to steroid therapy.

Key Words: Hydronephrosis, Retroperitoneal fibrosis, Steroids