Retroperitoneal Fibrosis Treatment & Management

  • Author: Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU; Chief Editor: Bradley Fields Schwartz, DO, FACS   more...
 
Updated: Jan 19, 2012
 

Medical Care

Optimal care in patients with retroperitoneal fibrosis (RPF) requires an integrated approach of surgical and nonsurgical therapies. The aims of management are to preserve renal function, to prevent other organ involvement, to exclude malignancy, and to relieve symptoms. The literature reports no consensus on the appropriate management of patients with retroperitoneal fibrosis because no controlled therapeutic trials have been performed. Furthermore, successful outcome has occasionally been reported with conservative therapy.[44, 45] In 2002, Pistolese et al reported partial or complete regression of retroperitoneal fibrosis associated with inflammatory aortic aneurysm after surgery.[5] The treatment of retroperitoneal fibrosis depends on the stage of the disease at diagnosis, as depicted in the image below.

Management algorithm of retroperitoneal fibrosis. Management algorithm of retroperitoneal fibrosis.

Empirical therapy includes corticosteroids, tamoxifen, and azathioprine; experimental therapy includes azathioprine, cyclophosphamide, mycophenolate-mofetil, cyclosporin, medroxyprogesterone acetate, and progesterone. Glucocorticoids and azathioprine are most useful in patients with signs of inflammation (eg, raised ESR and WBC count and positive ANA results).

  • Corticosteroids
    • In 1958, Ross and Tinckler first reported the use of corticosteroids in the treatment of retroperitoneal fibrosis. The beneficial effect is thought to be due to anti-inflammatory action and the ability to inhibit fibrotic tissue maturation.
    • A pooled analysis of nonmalignant retroperitoneal fibrosis treated with steroids revealed a satisfactory outcome. In 2002, van Bommel analyzed 147 patients and noted good results in 122 patients (83%) and recurrence in 55 patients (16%). Most recurrences were noted within 12 months, and some responded to reintroduction of steroid.[46] Despite their proven success, using steroids as a first-line therapy in retroperitoneal fibrosis remains controversial because many clinicians believe that multiple deep biopsies are still essential to exclude malignancy.
    • A standard protocol is prednisolone at 40-60 mg/d tapered to 10 mg/d within 2-3 months and discontinued after 12-24 months. Timely dose reductions and cessation are important because of the adverse effects associated with long-term steroid use.
    • In 1994, Harreby et al used methylprednisolone pulse therapy (MPPT) at 1 g/d IV for 3 days along with azathioprine or penicillamine. This therapy was used in 11 cases of retroperitoneal fibrosis with ureteric obstruction following initial insertion of ureteral stents. The treatment was successful in 7 patients but only moderately effective in 4 patients. The combination of glucocorticoid and azathioprine is most useful in patients with signs of inflammation (raised ESR, positive ANA results, positive PET findings).[47]
    • Steroids can be used in combination with surgery. In one study, concomitant use of steroids with surgery reduced the rate of ureteric restenosis from 48% to 10%.[48] However, response may vary, and an unacceptably high dose of steroid may be required to control retroperitoneal fibrosis.
    • Complications of corticosteroid treatment include obesity, Cushingoid features, striae, retarded growth, and an increased susceptibility to infections, hypertension, osteoporosis, cataracts, peptic ulcer disease, and diabetes mellitus.
  • Tamoxifen
    • In 1991, Clarke et al were the first to use tamoxifen, a nonsteroidal antiestrogen, in the treatment of retroperitoneal fibrosis.[49]
    • Its mechanism of action is not entirely clear, and different hypotheses have been proposed. Tamoxifen increases the synthesis and secretion of transforming growth factor–beta (TGF-b), an inhibitory growth factor, by human fetal fibroblast in vitro. In retroperitoneal fibrosis, fibroblast and immune cells in the inflammatory mass may increase their secretion of TGF-b, which may then decrease the size of the fibrous plaque.[50] Other possible mechanisms of action include inhibition of protein kinase C, reduction of epidermal growth factor production, inhibition of calmodulin, and blockage of growth-promoting histaminelike receptor.[51, 52]
    • Clinicians have reported successful treatment with tamoxifen. Various authors have used tamoxifen with a variable protocol (10-40 mg for 6 mo to 3 y).
    • In a randomized, open-label, controlled trial, Vaglio et al compared the efficacy of prednisone with that of tamoxifen in maintaining remission among patients with idiopathic retroperitoneal fibrosis. A total of 40 patients (18-85 y) were enrolled in the study. Following induction therapy with 1 mg/kg daily of prednisone for 1 month, those patients who had achieved remission were randomly assigned to receive tapering prednisone (initial dose 0·5 mg/kg daily) for 8 months or tamoxifen (fixed dose 0·5 mg/kg daily) for 8 months. After 18 months, the authors found the cumulative relapse probability was estimated at 17% with prednisone and 50% with tamoxifen (difference -33% [-62 to -3, p=0·0372]). However, Cushingoid changes and grade 2 hypercholesterolemia were more common in the prednisone group than in the tamoxifen group (p=0·0116 and p=0·0408, respectively).[53]
    • Compared with steroids, the adverse effect profile of tamoxifen is low; thus, clinicians consider tamoxifen a reasonable treatment option. However, the adverse effects of tamoxifen, especially an increased risk of thromboembolism and ovarian cancer, should be carefully considered for each patient.
  • Mycophenolate mofetil
    • To block the proliferation of T cells and B cells, a combination of mycophenolate mofetil (MMF) and steroid appears to be a promising option.
    • Swartz et al (2008) reported their experience with high-dose corticosteroid and MMF in 21 patients. They used prednisone 60 mg or 120 mg qid for 3-6 months and tapered the dose upon clinical improvement. In addition, steroid-sparing therapy with MMF (1000 mg bid) was recommended, with discontinuation after 6-12 months of therapy.[54]
    • In a recent study, Adler et al (2008) observed radiologic regression following therapy with MMF 2 g/d and prednisolone 1 mg/kg in 9 patients. Therapy led to removal of the ureteral catheter in 5 of 7 patients. Complications of MMF therapy included recurrent urinary infections and upper gastrointestinal disturbance.[55]
  • Azathioprine: Azathioprine has been used when steroid therapy has failed and as a steroid-sparing drug. Cogan and Fastrez used a 6-week course of azathioprine (150 mg/d) in a patient whose condition recurred soon after prednisone treatment was discontinued. They observed a significant response with azathioprine.[56] McDougal and MacDonell reported successful outcome in combination with prednisolone in a 14-year-old girl.[16]
  • Experimental therapy: More recently, immunosuppressive agents such as azathioprine, cyclophosphamide, MMF, methotrexate, and cyclosporin have been used to treat retroperitoneal fibrosis. Future steroid-sparing options could include anti-inflammatory drugs such as tumor necrosis factor-alpha (TNF-a) inhibitors and anti-CD20 drugs.[16, 57, 56, 58, 59]
  • Medroxyprogesterone acetate: In vitro, medroxyprogesterone acetate inhibits fibroblastic proliferation. Use of progesterone and medroxyprogesterone acetate as an alternative treatment has been reported with successful outcome.[60, 61]
Next

Surgical Care

  • Temporizing maneuvers in the form of percutaneous nephrostomy or ureteral stenting are recommended in the presence of obstructive uropathy.
  • Primary management of retroperitoneal fibrosis consists of open biopsy, ureterolysis, and lateral/intraperitoneal transposition or omental wrapping of the involved ureter, as depicted in the image below.Postureterolysis intravenous urogram demonstrates Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.
  • Open ureterolysis, although effective in 90% of patients, is associated with significant morbidity (60%) and mortality (9%) rates.[62, 63]
  • In the past 10 years, the use of laparoscopic surgery has expanded to include complex ablative and reconstructive procedures. Kavoussi first described laparoscopic ureterolysis for retroperitoneal fibrosis in 1992.[64] Since then, a few authors have reported successful laparoscopic ureterolysis with a more rapid recovery and a shorter hospital stay. Although the success rate is no better than that of open ureterolysis, the laparoscopic technique has the advantage of reducing mean hospital stay, use of analgesia, convalescence period, and morbidity.[64, 65, 66]
  • Styn et al compared open ureterolysis (12 patients) with laparoscopic ureterolysis (13 patients) and reported a significantly shorter hospital stay (open 5.9 d vs laparoscopic 2.1 d; p=.004).[67] The complication rates did not differ between the groups. The success rate was 87.5% after open ureterolysis and 93.8% after laparoscopic ureterolysis (p=1).
  • More recently, with the advancement of technology, cases of endourologic treatment of retroperitoneal fibrosis via percutaneous balloon dilatation or endoscopic incision, dilatation, and permanent wall stent have been reported, with varying results.[68]
  • Long-term ureteral stenting is a reasonable approach in high-risk and elderly patients. Ureteral stenting may be placed on a long-term basis (months to years) in order to bypass ureteral obstruction. Short-term stenting (weeks to months) may be used as an adjunct to open surgical procedures.
  • Other newer innovative surgical techniques have been described, such as ureterolysis and wrapping with Gore-Tex (GSM, WL Gore & Associates, Flagstaff, Ariz), excision of the ureter and reanastomosis, posterior preperitoneal flap, and renal autotransplantation.[69]
Previous
Next

Consultations

Patients with renal failure should be referred to a nephrologist early in the course of their disease and have continued nephrologic follow-up.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU  Consulting Urologist, Department of Urology, Pinderfields General Hospital, The Mid-Yorkshire Hospitals NHS Trust, UK

Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU is a member of the following medical societies: British Association of Urological Surgeons, British Medical Association, European Association of Urology, and International College of Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Joby Taylor, MBChB, FRCS  Consultant Urologist, Forth Valley Royal Hospital, UK

Joby Taylor, MBChB, FRCS is a member of the following medical societies: British Association of Urological Surgeons, European Association of Urology, International Continence Society, Royal College of Surgeons of Edinburgh, and United Kingdom Continence Society

Disclosure: Nothing to disclose.

Anthony J Browning, MB, ChB, FRCS(Edin)  Consultant Urological Surgeon, Mid Yorkshire NHS Trust, Pinderfields General Hospital; Associate Post Graduate Dean, Yorkshire and Humber Deanery, UK

Anthony J Browning, MB, ChB, FRCS(Edin) is a member of the following medical societies: British Association of Urological Surgeons, Endourological Society, and European Association of Urology

Disclosure: Nothing to disclose.

Specialty Editor Board

Martha K Terris, MD, FACS  Professor, Department of Surgery, Section of Urology, Director, Urology Residency Training Program, Medical College of Georgia; Professor, Department of Physician Assistants, Medical College of Georgia School of Allied Health; Chief, Section of Urology, Augusta Veterans Affairs Medical Center

Martha K Terris, MD, FACS is a member of the following medical societies: American Cancer Society, American College of Surgeons, American Institute of Ultrasound in Medicine, American Society of Clinical Oncology, American Urological Association, Association of Women Surgeons, New York Academy of Sciences, Society of Government Service Urologists, Society of University Urologists, Society of Urology Chairpersons and Program Directors, and Society of Women in Urology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Martin I Resnick, MD †  Former Lester Persky Professor and Chair, Department of Urology, Former Professor, Department of Oncology, Case Western Reserve University School of Medicine

Martin I Resnick, MD † is a member of the following medical societies: American College of Surgeons, American Federation for Medical Research, American Institute of Ultrasound in Medicine, American Medical Association, American Society for Bone and Mineral Research, American Society for Reproductive Medicine, American Society of Andrology, American Surgical Association, American Urological Association, Association for Academic Surgery, Endocrine Society, National Kidney Foundation, Ohio Urological Society, and Pan American Medical Association

Disclosure: Nothing to disclose.

J Stuart Wolf Jr, MD, FACS  The David A Bloom Professor of Urology, Director, Division of Endourology and Stone Disease, Department of Urology, University of Michigan Medical School

J Stuart Wolf Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, Society of University Urologists, and Society of Urologic Oncology

Disclosure: Nothing to disclose.

Chief Editor

Bradley Fields Schwartz, DO, FACS  Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, and Society of University Urologists

Disclosure: Nothing to disclose.

References

  1. Katz SM, Bates O, Yudis M, et al. Immune complex glomerulonephritis in a case of retroperitoneal fibrosis. Am J Clin Pathol. May 1977;67(5):436-9. [Medline].

  2. Carton RW, Wong R. Multifocal fibrosclerosis manifested by vena caval obstructions and associated with vasculitis. Ann Intern Med. Jan 1969;70(1):81-6. [Medline].

  3. Lepor H, Walsh PC. Idiopathic retroperitoneal fibrosis. J Urol. Jul 1979;122(1):1-6. [Medline].

  4. Mitchinson MJ. Chronic periaortitis and periarteritis. Histopathology. Jul 1984;8(4):589-600. [Medline].

  5. Pistolese GR, Ippoliti A, Mauriello A, et al. Postoperative regression of retroperitoneal fibrosis in patients with inflammatory abdominal aortic aneurysms: evaluation with spiral computed tomography. Ann Vasc Surg. Mar 2002;16(2):201-9. [Medline].

  6. Higgins PM, Aber GM. Idiopathic retroperitoneal fibrosis--an update. Dig Dis. 1990;8(4):206-22. [Medline].

  7. Hatsiopoulou O, Irving S, Sharma SD. Retroperitoneal fibrosis in 2 brothers. J Urol. Jan 2001;165(1):182. [Medline].

  8. Astudillo L, Alric L, Jamard B, Laroche M. [Retroperitoneal fibrosis in an HLA-B27-positive patient]. Rev Med Interne. Dec 1999;20(12):1149-50. [Medline].

  9. Neild GH, Rodriguez-Justo M, Wall C, Connolly JO. Hyper-IgG4 disease: report and characterisation of a new disease. BMC Med. Oct 6 2006;4:23. [Medline].

  10. Uibu T, Oksa P, Auvinen A, Honkanen E, Metsärinne K, Saha H, et al. Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet. May 1 2004;363(9419):1422-6. [Medline].

  11. Higgins PM, Bennett-Jones DN, Naish PF, Aber GM. Non-operative management of retroperitoneal fibrosis. Br J Surg. Jun 1988;75(6):573-7. [Medline].

  12. Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol Clin North Am. Nov 1996;34(6):1259-75. [Medline].

  13. Inaraja L, Franquet T, Caballero P, et al. CT findings in circumscribed upper abdominal idiopathic retroperitoneal fibrosis. J Comput Assist Tomogr. Nov-Dec 1986;10(6):1063-4. [Medline].

  14. Hulnick DH, Chatson GP, Megibow AJ, et al. Retroperitoneal fibrosis presenting as colonic dysfunction: CT diagnosis. J Comput Assist Tomogr. Jan-Feb 1988;12(1):159-61. [Medline].

  15. Arrive L, Hricak H, Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis: differentiation with MR imaging. Radiology. Jul 1989;172(1):139-43. [Medline].

  16. McDougal WS, MacDonell RC. Treatment of idiopathic retroperitoneal fibrosis by immunosuppression. J Urol. Jan 1991;145(1):112-4. [Medline].

  17. Dedeoglu F, Rose CD, Athreya BH, et al. Successful treatment of retroperitoneal fibrosis with tamoxifen in a child. J Rheumatol. Jul 2001;28(7):1693-5. [Medline].

  18. Buff DD, Bogin MB, Faltz LL. Retroperitoneal fibrosis. A report of selected cases and a review of the literature. N Y State J Med. Sep 1989;89(9):511-6. [Medline].

  19. Gilkeson GS, Allen NB. Retroperitoneal fibrosis. A true connective tissue disease. Rheum Dis Clin North Am. Feb 1996;22(1):23-38. [Medline].

  20. Koep L, Zuidema GD. The clinical significance of retroperitoneal fibrosis. Surgery. Mar 1977;81(3):250-7. [Medline].

  21. Birnberg FA, Vinstein AL, Gorlick G, et al. Retroperitoneal fibrosis in children. Radiology. Oct 1982;145(1):59-61. [Medline].

  22. van Bommel EF, van Spengler J, van der Hoven B, Kramer P. Retroperitoneal fibrosis: report of 12 cases and a review of the literature. Neth J Med. Dec 1991;39(5-6):338-45. [Medline].

  23. Thomas MH, Chisholm GD. Retroperitoneal fibrosis associated with malignant disease. Br J Cancer. Nov 1973;28(5):453-8. [Medline].

  24. Zabetakis PM, Novich RK, Matarese RA, Michelis MF. Idiopathic retroperitoneal fibrosis: a systemic connective tissue disease?. J Urol. Jul 1979;122(1):100-2. [Medline].

  25. Izzedine H, Servais A, Launay-Vacher V, Deray G. Retroperitoneal fibrosis due to Wegener's granulomatosis: a misdiagnosis as tuberculosis. Am J Med. Aug 1 2002;113(2):164-6. [Medline].

  26. Barrison IG, Walker JG, Jones C, Snell ME. Idiopathic retroperitoneal fibrosis--is serum alkaline phosphatase a marker of disease activity?. Postgrad Med J. Mar 1988;64(749):239-41.

  27. Vaglio A, Corradi D, Manenti L, Ferretti S, Garini G, Buzio C. Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med. Apr 15 2003;114(6):454-62. [Medline].

  28. Webb AJ, Dawson-Edwards P. Non-malignant retroperitoneal fibrosis. Br J Surg. Jun 1967;54(6):508-18. [Medline].

  29. Saldino RM, Palubinskas AJ. Medial placement of the ureter: a normal variant which may simulate retroperitoneal fibrosis. J Urol. Apr 1972;107(4):582-5. [Medline].

  30. Clouse ME, Fraley EE, Litwin SB. Lymphangiographic criteria for diagnosis of retroperitoneal fibrosis. Radiology. Jul 1964;83:1-5.

  31. Rubenstein WA, Gray G, Auh YH, et al. CT of fibrous tissues and tumors with sonographic correlation. AJR Am J Roentgenol. Nov 1986;147(5):1067-74. [Medline].

  32. Amis ES Jr. Retroperitoneal fibrosis. AJR Am J Roentgenol. Aug 1991;157(2):321-9. [Medline].

  33. Barker CD, Brown JJ. MR imaging of the retroperitoneum. Top Magn Reson Imaging. Spring 1995;7(2):102-11. [Medline].

  34. Burn PR, Singh S, Barbar S, et al. Role of gadolinium-enhanced magnetic resonance imaging in retroperitoneal fibrosis. Can Assoc Radiol J. Jun 2002;53(3):168-70. [Medline].

  35. Liebman RM. Positive gallium scan in retroperitoneal fibrosis. AJR Am J Roentgenol. Nov 1983;141(5):949-50. [Medline].

  36. Kubota K, Yamada K, Yoshioka S, et al. Differential diagnosis of idiopathic fibrosis from malignant lymphadenopathy with PET and F-18 fluorodeoxyglucose. Clin Nucl Med. May 1992;17(5):361-3. [Medline].

  37. Vaglio A, Versari A, Fraternali A. (18)F-fluorodeoxyglucose positron emission tomography in the diagnosis and followup of idiopathic retroperitoneal fibrosis. Arthritis Rheum. Feb 15 2005;53(1):122-5.

  38. Dash RC, Liu K, Sheafor DH, Dodd LG. Fine-needle aspiration findings in idiopathic retroperitoneal fibrosis. Diagn Cytopathol. Jul 1999;21(1):22-6. [Medline].

  39. Pfammatter T, Hilfiker PR, Kurrer M, Sulser T. Transcaval biopsy of retroperitoneal fibrosis. Urol Int. Aug 1998;60(4):258-61. [Medline].

  40. Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol. 1948;59:1072-9.

  41. Mitchinson MJ. The pathology of idiopathic retroperitoneal fibrosis. J Clin Pathol. Nov 1970;23(8):681-9. [Medline].

  42. Corradi D, Maestri R, Palmisano A, Bosio S, Greco P, Manenti L, et al. Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. Kidney Int. Sep 2007;72(6):742-53. [Medline].

  43. Wu J, Catalano E, Coppola D. Retroperitoneal fibrosis (Ormond's disease): clinical pathologic study of eight cases. Cancer Control. Sep-Oct 2002;9(5):432-7. [Medline].

  44. Adam U, Mack D, Forstner R, Fritzenwallner A, Frick J. Conservative treatment of acute Ormond's disease. Tech Urol. Mar 1999;5(1):54-6. [Medline].

  45. Kume H, Kitamura T. Spontaneous regression of bilateral hydronephrosis due to retroperitoneal fibrosis. Scand J Urol Nephrol. Jun 2001;35(3):255-6. [Medline].

  46. van Bommel EF. Retroperitoneal fibrosis. Neth J Med. Jul 2002;60(6):231-42. [Medline].

  47. Harreby M, Bilde T, Helin P. Retroperitoneal fibrosis treated with methylprednisolone pulse and disease-modifying antirheumatic drugs. Scand J Urol Nephrol. Sep 1994;28(3):237-42. [Medline].

  48. Wagenknecht LV, Hardy JC. Value of various treatments for retroperitoneal fibrosis. Eur Urol. 1981;7(4):193-200. [Medline].

  49. Clark CP, Vanderpool D, Preskitt JT. The response of retroperitoneal fibrosis to tamoxifen. Surgery. Apr 1991;109(4):502-6. [Medline].

  50. Spillane RM, Whitman GJ. Treatment of retroperitoneal fibrosis with tamoxifen. AJR Am J Roentgenol. Feb 1995;164(2):515-6. [Medline].

  51. Horgan K, Cooke E, Hallett MB, Mansel RE. Inhibition of protein kinase C mediated signal transduction by tamoxifen. Importance for antitumour activity. Biochem Pharmacol. Dec 15 1986;35(24):4463-5. [Medline].

  52. Loffeld RJ, van Weel TF. Tamoxifen for retroperitoneal fibrosis. Lancet. Feb 6 1993;341(8841):382. [Medline].

  53. Vaglio A, Palmisano A, Alberici F, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet. Jul 23 2011;378(9788):338-46. [Medline].

  54. Swartz RD, Lake AM, Roberts WW, Faerber GJ, Wolf JS Jr. Idiopathic retroperitoneal fibrosis: a role for mycophenolate mofetil. Clin Nephrol. Apr 2008;69(4):260-8. [Medline].

  55. Adler S, Lodermeyer S, Gaa J, Heemann U. Successful mycophenolate mofetil therapy in nine patients with idiopathic retroperitoneal fibrosis. Rheumatology (Oxford). Oct 2008;47(10):1535-8. [Medline].

  56. Cogan E, Fastrez R. Azathioprine. An alternative treatment for recurrent idiopathic retroperitoneal fibrosis. Arch Intern Med. Apr 1985;145(4):753-5. [Medline].

  57. Marzano A, Trapani A, Leone N, et al. Treatment of idiopathic retroperitoneal fibrosis using cyclosporin. Ann Rheum Dis. Apr 2001;60(4):427-8. [Medline].

  58. Scavalli AS, Spadaro A, Riccieri V, et al. Long-term follow-up of low-dose methotrexate therapy in one case of idiopathic retroperitoneal fibrosis. Clin Rheumatol. Jul 1995;14(4):481-4. [Medline].

  59. Kaipiainen-Seppanen O, Jantunen E, Kuusisto J, Marin S. Retroperitoneal fibrosis with antineutrophil cytoplasmic antibodies. J Rheumatol. Apr 1996;23(4):779-81. [Medline].

  60. Barnhill D, Hoskins W, Burke T, et al. The treatment of retroperitoneal fibromatosis with medroxyprogesterone acetate. Obstet Gynecol. Sep 1987;70(3 Pt 2):502-4.

  61. Comini Andrada E, Hoschoian JC, Anton E, Lanari A. Growth inhibition of fibroblasts by progesterone and medroxyprogesterone in vitro. Int Arch Allergy Appl Immunol. 1985;76(2):97-100. [Medline].

  62. Kerr WS, Suby HI, Vickery A, Fraley E. Idiopathic retroperitoneal fibrosis: clinical experiences with 15 cases, 1956-1967. J Urol. May 1968;99(5):575-84. [Medline].

  63. Miles RM, Brock J, Martin C. Idiopathic retroperitoneal fibrosis. A sometime surgical problem. Am Surg. Feb 1984;50(2):76-84. [Medline].

  64. Kavoussi LR, Clayman RV, Brunt LM, Soper NJ. Laparoscopic ureterolysis. J Urol. Feb 1992;147(2):426-9. [Medline].

  65. Matsuda T, Arai Y, Muguruma K, et al. Laparoscopic ureterolysis for idiopathic retroperitoneal fibrosis. Eur Urol. 1994;26(4):286-90. [Medline].

  66. Fugita OE, Jarrett TW, Kavoussi P, Kavoussi LR. Laparoscopic treatment of retroperitoneal fibrosis. J Endourol. Oct 2002;16(8):571-4. [Medline].

  67. Styn NR, Frauman S, Faerber GJ, Wolf JS Jr. University of Michigan surgical experience with ureterolysis for retroperitoneal fibrosis: a comparison of laparoscopic and open surgical approaches. Urology. Feb 2011;77(2):339-43. [Medline].

  68. Slavis SA, Wilson RW, Jones RJ, Swift C. Long-term results of permanent indwelling wallstents for benign mid-ureteral strictures. J Endourol. Sep 2000;14(7):577-81. [Medline].

  69. Safioleas M, Safioleas P, Stamatakos M, Safioleas C. Retroperitoneal fibrosis obstructing the ureter: a new technique to prevent stenosis recurrence. Surgery. Feb 2008;143(2):299-300. [Medline].

  70. Baker LR. Auto-allergic periaortitis (idiopathic retroperitoneal fibrosis). BJU Int. Nov 2003;92(7):663-5. [Medline].

  71. Cosbie Ross J, Tinckler LF. Renal failure due to peri-ureteric fibrosis. Br J Surg. Jul 1958;46(195):58-62. [Medline].

  72. Cronin CG, Lohan DG, Blake MA, Roche C, McCarthy P, Murphy JM. Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol. Aug 2008;191(2):423-31. [Medline].

  73. Docimo SG, Dewolf WC. High failure rate of indwelling ureteral stents in patients with extrinsic obstruction: experience at 2 institutions. J Urol. Aug 1989;142(2 Pt 1):277-9. [Medline].

  74. Elashry OM, Nakada SY, Wolf JS, et al. Ureterolysis for extrinsic ureteral obstruction: a comparison of laparoscopic and open surgical techniques. J Urol. Oct 1996;156(4):1403-10. [Medline].

  75. Khan AN, Chandramohan M, Macdonald S. Retroperitoneal fibrosis. Available at: http://emedicine.medscape.com/article/380772-overview. eMedicine Journal [serial online]. 2002;[Full Text].

  76. Miller OF, Smith LJ, Ferrara EX, et al. Presentation of idiopathic retroperitoneal fibrosis in the pediatric population. J Pediatr Surg. Nov 2003;38(11):1685-8. [Medline].

  77. Parums DV, Choudhury RP, Shields SA, Davies AH. Characterisation of inflammatory cells associated with "idiopathic retroperitoneal fibrosis". Br J Urol. Jun 1991;67(6):564-8. [Medline].

  78. Albarran J. Retention renale par periureterite. Liberation externe de l'uretere. Association francaise d'urologie. 1905;9:511.

  79. Bourouma R, Chevet D, Michel F, et al. Treatment of idiopathic retroperitoneal fibrosis with tamoxifen. Nephrol Dial Transplant. Nov 1997;12(11):2407-10. [Medline].

 
Previous
Next
 
Intravenous urogram shows medial deviation of the middle part of both ureters.
Retrograde ureterogram reveals smooth narrowing and medial shift of the ureter.
Retrograde pyelogram demonstrates hydronephrosis.
Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.
Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.
Management algorithm of retroperitoneal fibrosis.
Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.
Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.
Abdominal radiograph demonstrates a wall stent on the right side.
Table. Differential Diagnoses of Retroperitoneal Fibrosis[42]
Retroperitoneal FibrosisRetroperitoneal LymphomaSclerosing MesenteritisDesmoid-Type FibromatosisInflammatory Myofibroblastic TumorWell-Differentiated Liposarcoma Sclerosing Variant
Ureteral displacementMedialLateral
Ureteral obstruction~80%~50%RareRareRareUnknown
Aortic displacementRareAnterior
Reactive perivascular lymphoid aggregates100%AbsentVariableRareVariablePresent in the inflammatory type
NecrosisAbsentVariableFat necrosisRareFocalFat necrosis
Vasculitis~50%AbsentAbsentAbsentAbsentAbsent
ClonalityAbsentVariableAbsentAbsentAbsentPresent
Β-cateninNegativeUnknownNegativePositive in 90% of casesNegativeVariable positivity
ALK-1NegativeUsually negativeNegativeNegativePositive in 50% of casesNegative
CD-117Negative in spindle cell componentRareVariableNegativeRareNegative
DesminNegativeNegativeVariableRareUsually positiveRare
S100NegativeNegativeNegativeRareNegativeUsually positive in the adipocytic component
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.