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Retroperitoneal Fibrosis

  • Author: Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
 
Updated: Jun 21, 2016
 

Practice Essentials

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 and resulting in entrapment and obstruction of retroperitoneal structures, notably the ureters. See the image below.

Intravenous urogram shows medial deviation of the Intravenous urogram shows medial deviation of the middle part of both ureters.

Signs and symptoms

The symptoms and signs associated with RPF are nonspecific, including the following:

  • Dull, poorly localized, noncolicky pain in the flank, back, scrotum, or lower abdomen [1] : Present in 92% of cases; in children, pain may be referred to the ipsilateral hip or gluteal region, resulting in resistance to hip extension [2]
  • Fever
  • Lower-extremity edema
  • Phlebitis
  • Deep venous thrombosis
  • Weight loss, nausea, vomiting, anorexia, and malaise: Uncommon presentations
  • Raynaud phenomenon, ureteric colic, hematuria, claudication, and urinary frequency: Rare manifestations

RPF can also be associated with Crohn disease, ulcerative colitis, and sclerosing cholangitis.

See Clinical Presentation for more detail.

Diagnosis

The diagnosis of RPF requires a high degree of suspicion, and the evaluation of patients with presumed idiopathic RPF includes ruling out secondary RPF due to malignancy, infection, retroperitoneal injury, or drugs.

Examination in patients with retroperitoneal fibrosis includes the following findings:

  • Hypertension: Present in approximately 50% of affected individuals with RPF
  • Presence of an abdominal mass (occasionally)
  • Obstruction of the ureters, with varying degree of renal insufficiency: Earliest and most common organ involvement

Uncommon physical findings due to late complications include the following:

  • Ascites
  • Peripheral edema or thrombosis
  • Hydrocele
  • Jaundice
  • Small- or large-bowel obstruction
  • Spinal cord compression

Testing

Laboratory studies and potential results for patients with suspected RPF include the following:

  • Erythrocyte sedimentation rate level: Elevated
  • C-reactive protein level: Elevated
  • Urea and creatinine levels: Elevated
  • Complete blood count: Normocytic normochromic anemia
  • Alkaline phosphatase levels (also reported as a marker [3] )
  • Antinuclear antibody levels (present in 60% of cases) [4]
  • Urinalysis: Usually normal; rarely, microscopic hematuria or pyuria is seen
  • Plasma electrophoresis: Polyclonal hypergammaglobulinemia

Imaging tests

The following imaging studies may be used to evaluate RPF:

  • Plain chest and abdominal radiography
  • Intravenous urography
  • Retrograde pyelography
  • Lymphangiography
  • Kidney ultrasonography
  • Abdominal CT scanning: Most frequently used imaging method for diagnosis and follow-up of RPF
  • Abdominal MRI
  • Nuclear imaging (eg, 67 Ga, 18 FDG): Very limited role
  • Positron emission tomography

Procedures

Biopsy and histologic/immunohistochemical examination of the affected tissue can aid in the diagnosis and determination of the extent of the disease, which in turn affect treatment management strategies. Open biopsy can ensure a definite histologic diagnosis, but it is associated with significant morbidity. Laparoscopic biopsy is safe, minimally invasive, cost-effective, and useful in making therapeutic decisions for retroperitoneal masses.

See Workup for more detail.

Management

There is currently no consensus on the appropriate management of patients with RPF, because no controlled therapeutic trials have been performed, and conservative therapy has occasionally yielded successful outcomes.[5, 6]

Optimal care in patients with RPF requires an integrated approach of surgical and nonsurgical therapies, and it is dependent on the stage of the disease at diagnosis. The aims of management include the following:

  • Preserve renal function
  • Prevent other organ involvement
  • Exclude malignancy
  • Relieve symptoms

Surgical option

Surgical ureterolysis has been the preferred primary mode of treatment for RPF, because it allows biopsy specimens to be obtained while ureteral obstruction is relieved. Thus, primary management of RPF consists of open biopsy, ureterolysis, and lateral/intraperitoneal transposition or omental wrapping of the involved ureter.

Other surgical interventions that may be involved in RPF include the following:

  • Temporizing maneuvers in the form of percutaneous nephrostomy or ureteral stenting: Recommended in the presence of obstructive uropathy
  • Laparoscopic surgery: Includes complex ablative and reconstructive procedures
  • Endourologic treatment via percutaneous balloon dilatation or endoscopic incision, dilatation, and permanent wall stent [7]
  • Long-term ureteral stenting: Reasonable approach in high-risk and elderly patients to bypass ureteral obstruction
  • Short-term ureteral stenting: Adjunct to open surgical procedures
  • Relatively newer innovative surgical techniques: Ureterolysis and wrapping with Gore-Tex, excision of the ureter and reanastomosis, posterior preperitoneal flap, and renal autotransplantation [8]

Pharmacotherapy

Medications used in the management of RPF include the following:

  • Glucocorticoids (eg, prednisolone, prednisone)
  • Immunosuppressive agents (eg, mycophenolate, azathioprine)
  • Estrogen receptor antagonists (eg, tamoxifen)

Empirical therapy includes corticosteroids, tamoxifen, and azathioprine. The possibility of autoallergic etiology prompted the use of corticosteroids and cytotoxic drugs in managing RPF. Glucocorticoids and azathioprine are most useful in patients with signs of inflammation.

See Treatment and Medication for more detail.

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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.[9, 10] Approximately 8% of cases are associated with metastatic malignancy.[11]

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.[12] The frequent association of retroperitoneal fibrosis with aortic aneurysm and the regression of fibrosis reported following aneurysm repair further supports this theory.[13] 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.[14] 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 that genetic factors may also play a role.[15] The human leukocyte antigen (HLA)–B27 cell marker has been demonstrated in several patients with retroperitoneal fibrosis.[16]

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.[17] 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.[18]

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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Frequency

International

Retroperitoneal fibrosis is a relatively uncommon disease. The estimated annual incidence varies from 1 per 200,000-500,000 population.[19, 20]

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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.[21, 22]

Malignant retroperitoneal fibrosis is associated with poor prognosis, and most patients have an average survival of approximately 3-6 months.[23] Idiopathic retroperitoneal fibrosis carries a good prognosis, with little effect on long-term morbidity or mortality.

Race-, Sex-, and Age-related Demographics

Retroperitoneal fibrosis has no reported racial predilection. The condition occurs twice as commonly in males as in females. The peak incidence of retroperitoneal fibrosis is in adults aged 40-60 years.[11, 24] Childhood presentation is extremely rare. To date, approximately 33 cases in children younger than 18 years have been reported.[25]

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Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Coauthor(s)

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: Endourological Society, British Association of Urological Surgeons, European Association of Urology

Disclosure: Nothing to disclose.

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

Joby Taylor, FRCS, MBChB 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, United Kingdom Continence Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Astellas.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, Society of Laparoendoscopic Surgeons, Society of University Urologists, Association of Military Osteopathic Physicians and Surgeons, American Urological Association, Endourological Society

Disclosure: Nothing to disclose.

Additional Contributors

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, Association of Women Surgeons, American Society of Clinical Oncology, Society of Urology Chairpersons and Program Directors, Society of Women in Urology, Society of Government Service Urologists, American College of Surgeons, American Institute of Ultrasound in Medicine, American Urological Association, New York Academy of Sciences, Society of University Urologists

Disclosure: Nothing to disclose.

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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 [40]
  Retroperitoneal Fibrosis Retroperitoneal Lymphoma Sclerosing Mesenteritis Desmoid-Type Fibromatosis Inflammatory Myofibroblastic Tumor Well-Differentiated Liposarcoma Sclerosing Variant
Ureteral displacement Medial Lateral        
Ureteral obstruction ~80% ~50% Rare Rare Rare Unknown
Aortic displacement Rare Anterior        
Reactive perivascular lymphoid aggregates 100% Absent Variable Rare Variable Present in the inflammatory type
Necrosis Absent Variable Fat necrosis Rare Focal Fat necrosis
Vasculitis ~50% Absent Absent Absent Absent Absent
Clonality Absent Variable Absent Absent Absent Present
Β-catenin Negative Unknown Negative Positive in 90% of cases Negative Variable positivity
ALK-1 Negative Usually negative Negative Negative Positive in 50% of cases Negative
CD-117 Negative in spindle cell component Rare Variable Negative Rare Negative
Desmin Negative Negative Variable Rare Usually positive Rare
S100 Negative Negative Negative Rare Negative Usually positive in the adipocytic component
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