Retroperitoneal fibrosis: Cologne experience in conservative and surgical management

Retroperitoneal fibrosis (RPF) is an uncommon inflammatory disease of the retroperitoneum leading to extensive fibrosis with consecutive obstruction of adjacent organs, namely the ureters. Since no consensus on the standard therapy exists, aim of the current study was to evaluate the outcome of 39 patients with RPF. Between 1986 and 1997 39 cases of RPF were diagnosed: 21 cases had primary RPF and 18 patients had secondary RPF after aortofemoral graft (n = 13), radiation (n = 2), or prior retroperitoneal surgery (n = 2). 21 patients demonstrated unilateral and 16 cases had bilateral hydronephrosis, in 2 patients no dilatation was observed. In 28 cases (n = 12 primary RPF, n = 16 secondary RPF) intial management consisted of DJ-stent placement, whereas in 11 cases(n = 9 primary RPF, n = 2 secondary RPF) percutaneous nephrostomy had to be placed. All patients received oral immunosuppressive agents (prednisolone 1 mg/kg, azathioprine 1 mg/kg/day) for 3 months before reevaluation was performed. In case of complete remission, immunosuppressive medication was continued for another 3 months, in case of stable disease or progression surgery was performed. In 26 cases (n = 15 primary RPF, n = 11 secondary RPF) ureterolysis with intraperitoneal displacement and omental wrapping was performed. 3 patients demonstrated complete remission after oral prednisolone/azathioprine; in 2 cases RPF presented as pelvic mass and was resected followed by immunsuppressive therapy, in another 2 cases bilateral ileal replacement of the ureters had to be performed and 4 cases remained on DJ-stents and nephrostomy, resp. Postoperatively, all patients with primary RPF were continued on immunsuppressive medication for another 3 months. After a follow-up of 6 to 120 months only 3 patients developed a retroperitoneal recurrence and were treated by unilateral nephrectomy or DJ stent placement (n = 2). Our data suggest that the combination of both immunosuppressive medication and surgical management results in an excellent longterm outcome in idiopathic retroperitoneal fibrosis with a recurrence rate of only 8%. Combination therapy should be considered as therapeutic option early in the course of the disease. Primary reconstructive surgery appears to be the most promising approach in secondary retroperitoneal fibrosis with a recurrence rate of only 5%; short external compression of the ureter might be managed by endoluminal balloon dilatation.