Symptoms

• The range of these symptoms can be mild to severe, with patients presenting with decreasing urine output and ultimately anuria, abdominal pain, lower extremity edema, and ischemic pain from arterial compression.

Diagnosis

• Intravenous urogram (IVU)
• Retrograde urethrogram
• Magnetic resonance imaging (MRI) and computed tomography (CT) scanning

Medical Management

• Urinary diversion can be initiated with placement of ureteral stents or double J catheters
• With failure to gain access via the retrograde approach, percutaneous placement of bilateral nephrostomy tubes or nephroureteral stents may be necessary
• Treatment with corticosteroids may halt the obstructive process and stabilize the ureteral obstruction

Surgical Management

• Exploratory laparotomy with appropriate biopsies to rule out cancer as the cause of fibrosis
• If the frozen sections exclude cancer then ureterolysis should be performed with dissection of the ureter from the plaque and followed by intraperitonealization and omental wrapping of the ureters should be performed
• Postoperative treatment with corticosteroids can be considered as part of the management of idiopathic RPF but their efficacy has never been proven

If ureterolysis must be abandoned, salvage techniques inclued:

• Ureteral-ileo segment interposition
• Ureteral reimplantation with a psoas hitch or Boari flaps
• Autotransplantation

Life-time follow up is necessary:

• Blood studies to monitor renal function
• Diagnostic imaging studies (renal ultrasound, renal scan, IVU, CT or MRI scanning)

References

• Baker LRI, Mallinson WJ, Gregory MC, et al: Idiopathic retroperitoneal fibrosis: A retrospective analysis of 60 cases. Br J Urol 60:497-503, 1988. Dalton RR, Donohue JH, Mucha P, et al: Management of retroperitoneal sarcomas. Surgery 105:725, 1989.
• Felix EL, Wood DK, DasGupta TK: Tumors of the retroperitoneum. Curr Probl Cancer 6:3, 1981.
• Koep L, Zuidema, GD: The clinical significance of retroperitoneal fibrosis. Surgery 82:250-257, 1977.
• Sheinfeld J, Eturk E, Spatano RF, et al: Peritoneal abscess:Current concepts. J Urol 127:191-194,1987.
• Wagenknect LV, Hardy JC: Value of various treatment for retroperitoneal fibrosis. Eur Urol 7:193-200, 1981.

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