Retroperitoneal fibrosis

Retroperitoneal fibrosis is a rare disease but is important surgically because it can produce ureteral obstruction and renal failure. A wide variety of inflammatory, infective and neoplastic conditions can result in secondary retroperitoneal fibrosis and it is essential to look carefully for an underlying neoplastic condition in all cases of retroperitoneal fibrosis. Idiopathic retroperitoneal fibrosis occurs predominantly in men and results in some form of renal failure in 75% of patients. The classic triad of medial deviation of ureters, ureteral narrowing at L4-5 and proximal hydronephrosis is usually seen in high-dose intravenous pyelograms and the ease of retrograde ureteral catheterization is often diagnostic. Management depends on the degree of renal impairment, with laparotomy, multiple biopsies and ureterolysis being the treatment of choice. Steroids are beneficial, if used early, and the prognosis is excellent if there is no underlying malignant condition.