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  • • Retroperitoneal fibrosis characterized by extensive fibrotic encasement of the retroperitoneal tissues

    • Most common in men over age 50 who have renal failure secondary to obstructive uropathy

    • Diffuse desmoplastic involvement of the retroperitoneum may alternatively give rise to obstructive jaundice or small or large bowel obstruction

    • Classic diagnostic triad includes:

    • -Bilateral hydronephrosis/hydroureter

      -Medial deviation of the ureters

      -Extrinsic ureteric compression at the L4-5 level

    • Over 67% of cases are idiopathic

    • Known etiologies include:

    • -Drugs

      -Inflammatory disorders

      -Retroperitoneal hemorrhage

      -Peri-aneurysmal (abdominal aortic aneurysm [AAA]) inflammation

      -Irradiation

      -Urinary extravasation

      -Cancer

    • Most common drugs associated with retroperitoneal fibrosis are methsergide and β-blockers

    • Most common inflammatory condition associated with retroperitoneal fibrosis is Sjögren syndrome

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Symptoms and Signs

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  • • Low back or flank pain

    • Symptoms of bowel obstruction

    • Jaundice (occasionally)

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Laboratory Findings

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  • • Uremia

    • Elevated creatinine

    • Pyuria

    • Microscopic hematuria

    • Elevated ESR and C-reactive protein levels

    • Elevated bilirubin (occasionally)

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Imaging Findings

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  • US: Demonstrates hydronephrosis

    CT scan or MRI: Demonstrates the classic findings that suggest the diagnosis:

    • -Fibrotic process

      -Bilateral hydronephrosis/ hydroureter

      -Medial deviation of the ureters

      -Extrinsic ureter compression

    • MRI more sensitive in differentiating between fibrosis and lymphoma or metastatic carcinoma

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  • • Retroperitoneal hematoma

    • Retroperitoneal abscess

    • Retroperitoneal sarcoma

    • Retroperitoneal teratoma

    • Metastatic disease

    • Lymphoma

    • Mesenteric lipodystrophy

    • Peritoneal mesothelioma

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Rule Out

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  • • AAA

    • Medication-induced fibrosis

    • Underlying malignancy, most commonly metastatic carcinoma or lymphoma

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  • • Complete history including risk factors and symptoms of systemic inflammatory diseases, such as Sjögren syndrome

    • Physical exam with thorough musculoskeletal evaluation

    • Radiographic characterization to evaluate for neoplasm and AAA

    • Percutaneous or operative biopsy of fibrotic mass for evidence of malignancy

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When to Admit

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  • • Most patients will have an obstructive uropathy that will require inpatient urinary decompression

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When to Refer

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  • • Urology for urinary decompression

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  • • Urinary decompression via ureteric stents or percutaneous nephrostomy

    • Repair of AAA if present

    • Discontinuation of suspect medications

    • Initiate anti-inflammatory medications

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Surgery

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Indications

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  • • Diagnostic laparoscopy with biopsy to rule out neoplasm and definitively establish the diagnosis of retroperitoneal fibrosis

    • Ureterolysis in select patients

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Medications

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  • • Prednisone and other immunosuppresants have been used with varying success

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Treatment Monitoring

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  • • Clinical resolution of urinary obstruction

    • Repeat imaging study

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Complications

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  • • Renal insufficiency/failure

    • Bowel obstruction

    • Failure of fibrosis to resolve requiring lifelong ureteral stents or percutaneous nephrostomy tubes

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Prognosis

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  • • Prognosis for gradual resolution is good as long as there is no underlying cancer

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