Retroperitoneal Fibrosis 

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

Background

Retroperitoneal fibrosis (RPF) is characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centered over the anterior surface of the fourth and fifth lumbar vertebrae. This fibrosis leads to entrapment and obstruction of retroperitoneal structures, notably the ureters. In most cases, the etiology is unknown. However, its occasional association with autoimmune diseases and its response to corticosteroids and immunosuppressive therapy suggest it is probably immunologically mediated.[1, 2] Approximately 8% of cases are associated with metastatic malignancy.[3]

The symptoms and signs associated with retroperitoneal fibrosis are nonspecific, and diagnosis requires a high degree of suspicion. Although a definitive diagnosis can only be made based on biopsy findings, intravenous urography may provide further support for the diagnosis of retroperitoneal fibrosis, particularly if the classic features are present. CT scanning or MRI is essential for evaluating the extent of the disease process.

The aims in management of retroperitoneal fibrosis include preserving renal function, reducing the morbidity, and suppressing inflammatory processes. Although most cases are idiopathic, precipitating causes should be excluded, particularly malignancy. Surgical ureterolysis has been the preferred primary mode of treatment because it allows biopsy specimens to be obtained while ureteral obstruction is relieved. Recently, the knowledge of a possible autoallergic etiology has prompted the use of corticosteroids and cytotoxic drugs.

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Pathophysiology

Anatomy of the retroperitoneum

The retroperitoneal space is bordered anteriorly by the posterior parietal peritoneum, posteriorly by the transversalis fascia, and superiorly by the diaphragm. Inferiorly, it extends to the level of pelvic brim. The anterior and posterior layers of renal fascia (Gerota fascia) subdivide the retroperitoneal space on either side of the spine into 3 compartments. The posterior space contains the pararenal fat. The intermediate space contains the kidney, the adrenal gland, and the perirenal fat. The anterior space is more extensive.

The anterior pararenal space is bordered anteriorly by the posterior parietal peritoneum, posteriorly by the anterior layer of renal fascia, and laterally by the lateral conal fascia. The anterior pararenal space contains the extraperitoneal portions of the ascending and descending colon, the duodenum, and the pancreas. The anterior pararenal space is continuous across the midline; however, collections of fluid tend to remain ipsilateral to the site of origin. Medially, the anterior layer of renal fascia blends with the connective tissue around the aorta and the inferior vena cava. The posterior layer fuses with the psoas fascia. Laterally, both layers merge to form lateral conal fascia.

Pathogenesis

Although the exact pathogenesis of retroperitoneal fibrosis has not been definitively described, good evidence supports the suggestion that it develops as an immunologic response to antigens within atherosclerotic plaques. Autopsy and CT studies have shown that fibrosis often begins around a severely atherosclerotic aorta. Thinning or breaching of the media may allow insoluble lipids, such as ceroid, an oxidized low-density lipoprotein, to leak into periaortic tissue, stimulating an immunologic reaction. This theory is supported by the presence of circulating anticeroid antibodies and the finding of ceroid-containing macrophages in nearby lymph nodes.

For this reason, Mitchinson suggested in a 1984 report that the condition should be termed chronic periaortitis.[4] The frequent association of retroperitoneal fibrosis with aortic aneurysm and the regression of fibrosis reported following aneurysm repair further supports this theory.[5] However, the occurrence of retroperitoneal fibrosis in children and those without an aneurysm suggests that other factors must be involved.

In some cases, an immune reaction to an external agent may initiate fibrosis. Drugs such as beta-blockers, methysergide, and methyldopa have been implicated, possibly by acting as haptens, leading to a hypersensitivity or autoimmune reaction.[6] The fibrous reaction associated with carcinoid tumor is believed to be the result of circulating serotonin or its metabolites. Methysergide is a strong serotonin antagonist, and the rebound release of serotonin following prolonged intake may be an alternate mechanism in this case. The association of retroperitoneal fibrosis with other connective-tissue diseases and reported familial occurrences suggest genetic factors may also play a role.[7] The human leukocyte antigen (HLA)–B27 cell marker has been demonstrated in several patients with retroperitoneal fibrosis.[8]

Recently, investigators have coined the term hyper–immunoglobulin G4 (hyper-IgG4) syndrome owing to raised levels of IgG4 and abundant IgG4-rich plasma cells in the inflammatory infiltrate.[9] Pathogenetic features of idiopathic retroperitoneal fibrosis include the following:

  • HLA-B27 or HLA-DRB1 03 haplotype
  • High serum IgG4 concentration
  • Raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level
  • Positive serum autoantibodies
  • Marked infiltration of IgG4-positive plasma cells

A 2004 case-control study increased that smoking and exposure to asbestos also increase the risk of retroperitoneal fibrosis.[10]

Ureteral obstruction in retroperitoneal fibrosis often appears minimal despite severe renal failure. This suggests that the obstruction relates to impairment of normal ureteric peristalsis by fibrotic tissue rather than to mechanical obstruction.

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Epidemiology

Frequency

International

Retroperitoneal fibrosis is a relatively uncommon disease. The estimated annual incidence varies from 1 per 200,000-500,000 population.[11, 12]

Mortality/Morbidity

Because of the nonspecific nature of the symptoms, the diagnosis of retroperitoneal fibrosis is often delayed. This may lead to progressive loss of renal function.

Envelopment of the inferior vena cava and lymphatics may result in compression or thrombosis and leads to lower-limb edema. In addition, involvement of gonadal vessels may cause scrotal edema. Occasionally, the duodenum, biliary tract, pancreas, large bowel, and mesentery are involved.[13, 14]

Malignant retroperitoneal fibrosis is associated with poor prognosis, and most patients have an average survival of approximately 3-6 months.[15] Idiopathic retroperitoneal fibrosis carries a good prognosis, with little effect on long-term morbidity or mortality.

Race

Retroperitoneal fibrosis has no reported racial predilection.

Sex

Retroperitoneal fibrosis is twice as common in males as in females.

Age

The peak incidence of retroperitoneal fibrosis is in adults aged 40-60 years.[3, 16] Childhood presentation is extremely rare. To date, approximately 33 cases in children younger than 18 years have been reported.[17]

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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].

 
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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
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