ureters

retropeitoneal fibrosis

Retroperitoneal fibrosis (RPF) is a rare condition where fibrous tissue forms in the back of the abdominal cavity near the kidneys, ureters, and major blood vessels. This can lead to the compression of these structures, potentially causing pain, kidney problems, and difficulty with urination. The exact cause is often unclear, but RPF can be linked to autoimmune conditions, certain medications, or infections. Early diagnosis is key, as untreated RPF can lead to serious complications. Treatment often involves medication to reduce inflammation and, in some cases, surgery to relieve pressure on affected organs.

////// indicates the sites often involved in RPF

retroperitoneal fibrosis (RPF)

Retroperitoneal fibrosis is an Inflammatory fibrotic process in the retroperitoneum causing compression of retroperitoneal structures, including ureters.

  • Incidence: 1 in 200,000

  • Peak age: 40-60 years

  • Gender prevalence: Males > females (2:1)

Aetiology

  • Idiopathic (70%): Possible immune response to ceroid, a polymer of oxidized lipids and protein from atherosclerotic plaques

  • Drugs:

    • Methysergide (migraine treatment)

    • LSD

    • Bromocriptine

    • Methyldopa

    • Beta blockers

    • Phenacetin

    • Amphetamine

  • Malignancy:

    • Lymphoma

    • Sarcoma

    • Carcinomas (breast, colon, stomach, prostate)

  • Infection:

    • Tuberculosis (TB)

    • Schistosomiasis

    • Chronic UTI

    • Syphilis

    • Gonorrhoea

    • Actinomycosis

  • Radiation exposure

  • Inflammatory conditions:

    • Inflammatory bowel disease (IBD)

    • Sarcoidosis

    • Endometriosis

    • Collagen diseases

    • Inflammatory abdominal aortic aneurysm (AAA)

    • Multifocal fibrosclerosis

the symptoms

Retroperitoneal fibrosis often presents with non-specific symptoms, leading to delayed diagnosis. Patients may experience general discomfort and signs related to compression of nearby structures.

  • Vague symptoms:

    • Back pain

    • Lethargy

    • Anorexia

    • Weight loss

  • Symptoms related to compression of structures:

    • Ureters: Oliguria (reduced urine output), weight gain, shortness of breath (SOB)

    • Inferior vena cava (IVC): Deep vein thrombosis (DVT)

    • Renal vein: Hypertension, haematuria (blood in urine)

What happens after referral

After receiving your referral, our practice nurse will triage your case, and you'll be promptly contacted for a brief overview of your condition. Preceding your visit, several diagnostic tests, including urine analysis, urine cytology, ultrasound, CT scan, and nuclear medicine test called MAG3, are arranged to enhance the evaluation. At your appointment, A/Prof Homi Zargar will engage with you, conduct a thorough assessment, and review the gathered information. A personalized care plan addressing your symptoms will be devised, ensuring all your questions are addressed.

Diagnosis of retroperitoneal fibrosis involves:

  • A comprehensive symptom assessment through a detailed history.

  • Urologists conducting examinations and ordering additional investigations, including

  • Midstream urine test to rule out infection or blood.

  • Imaging Studies:

    • Ultrasound: Non-invasive imaging to visualize the kidneys and ureters.

    • CT Urography (CVU): Contrast dye injected intravenously to enhance X-ray images of the urinary system.

    • Ureteroscopy: direct visualization of the ureter using a thin, flexible scope.

    • Biopsy: tissue sample analysis to identify potential malignancies.

      • If any malignancy is identified, the treatment algorithm changes significantly.

      • Biopsy considerations:

        • Cross-sectional imaging (CT/MRI) alone may not exclude malignancy, except in clear cases of lymphoma

        • Biopsy is recommended to rule out malignancy before proceeding with RPF treatment

        • Exceptions where biopsy might not be needed:

          • Classic features of RPF on imaging

          • No lymphadenopathy

          • No history of prior malignancy

      • Potential role of PET scanning:

        • FDG-PET can help exclude the need for biopsy, as lymphoma and sarcoma are both positive on PET scans

Treatment options

  • Individualized Approach: Recognizing the uniqueness of each case, A/Prof Homi Zargar tailors treatments specifically for your conditions and symptoms arising from RPF.

    Initial Management of Retroperitoneal Fibrosis

    • Decompression of urinary tracts :
      Immediate intervention may involve relieving urinary obstruction caused by fibrosis.

    • Primary treatment with high-dose steroids:

      • Effective in approximately 80% of cases, particularly in patients with raised ESR, leukocytosis, or inflammatory cells on biopsy.

      • Treatment duration is typically more than 6 months due to the risk of relapse, but some advocate for longer-term therapy.

    • Steroid-sparing immunosuppressants:

      • Medications like azathioprine, cyclosporine, mycophenolate mofetil, and tamoxifen have shown efficacy in some cases.

      • Non-steroidal immunosuppressants may prevent relapse after a short course of high-dose steroids.

Surgical Treatments:

  • The choice of surgical option depends on the location, length, and complexity of the ureteric involvement.

  • Stenting may serve as both a diagnostic tool and a temporary solution, particularly in cases where definitive surgery is delayed.

  • Stenting and endoscopic options :

    • Description: This minimally invasive procedure involves using a ureteroscope to visualize and incise the stricture, restoring normal ureteral diameter. Placement of a tube-like stent within the ureter to maintain patency and promote stricture healing.

    • Suitability: Temporary relief for proximal and distal obstruction; may serve as a bridge before definitive surgical intervention.

  • Open ureterolysis:

    • Involves a midline incision and medial mobilization of the left and right colon.

    • Ureters are dissected from the fibrous tissue and repositioned within the peritoneum or wrapped in the omentum to prevent recurrence of obstruction.

    • Biopsies are often taken during the procedure to rule out malignancy.

  • Robotic ureterolysis:

    • A minimally invasive technique that provides greater precision and control compared to traditional laparoscopy.

    • Ureters are pre-stented and carefully dissected from the surrounding fibrotic tissue using robotic instruments.

    • The ureters are then repositioned in the peritoneum or wrapped in omentum to prevent future obstruction.

    • Robotic surgery allows for improved visualization and dexterity, leading to fewer conversions to open surgery, and reduced complications.

    • Studies show a high success rate with most patients remaining free of obstruction long-term.