Editing Retroperitoneal Fibrosis (section)

== Management ==

* '''Decompression'''
** Patients with hydronephrosis and uremia should be emergently decompressed by either nephrostomy or ureteral stent
*** '''Ureteral stent placement is usually not difficult to perform in the setting of RPF.'''
*** '''Advantages of ureteral stents:'''
**** '''Opportunity to perform retrograde pyelograms to evaluate the anatomy'''
**** '''Convenience of internal drainage'''
*** In a critically ill patient with electrolyte abnormalities and little or no urine output, nephrostomy tube placement is favored.
** '''After decompression, the patient should be monitored closely for post-obstructive diuresis''', renal function status, and appropriate replacement of fluids and electrolytes.
* '''Medications'''
** '''Primary medical management involves steroid therapy'''
*** Patients who have evidence of active inflammation—manifested by increased ESR, CRP, leukocytosis, or active inflammation on a biopsy—are more likely to respond to steroid therapy.
** '''Use of immunosuppressive agents (azathioprine, mycophenolate mofetil, cyclosporine, cyclophosphamide, and colchicine) is reserved for patients in whom steroid therapy fails; relapses are as high as 50% during steroid tapering'''
** '''Other medications used: medroxyprogesterone acetate, progesterone, and particularly tamoxifen'''
* '''Ureterolysis'''
** May be performed open or laparoscopically
** '''Although hydronephrosis may be unilateral on preoperative assessment, in general the process is bilateral, requiring bilateral ureterolysis.'''
*** '''In bilateral ureterolysis, the ureters need to be protected by intraperitonealization or omental wrapping'''.
*** If ureterolysis is impossible to perform, renal autotransplantation may be performed