RETROPERITONEAL FIBROSIS

RETROPERITONEAL FIBROSIS


                RETROPERITONEAL FIBROSIS (ORMOND’S DISEASE)

  • RPF is an uncommon inflammatory condition characteristed by proliferation of fibrous tissue in the retroperitoneum.
  • Group of disorder associated with mediastinal fibrous, Duputyren’s contracture, plantar fasciitis, Peyronie’s disease, Riedel’s thyroiditis, sclerosing cholangitis, mesntric panniculitis, pseudotumour of orbit and fibromatosis.
  • The process usually begins at the level of aortic bifurcation and spreads cephalad upto renal artery.

 

BOUNDARIES-

  • Superior- dome of diaphragm
  • Inferior- Levator ani
  • Posterior- psoas muscle
  • Anterior- peritoneum

 

ETIOLOGY-

  • 70% cases are primary or idiopathic (Ormond’s fibrosis)
  • Secondary causes (30%)-

a) Inflammatory condition – CATH (Chronic pancreatitis, actinomycosis, TB, histoplasmosis)

b) Drugs- Methylsergide (5HT1 and 5HT2 receptor antagonist) , amphetamines

c) Malignancy- CA prostate, carcinoid tumours

d) Autoimmune disorder- SLE, PAN

e) Radiation

 

CLINICAL FEATURES-

  • Patient present with severe and persistent back pain
  • Males of 40- 60 years
  • Early symptoms are vague and non- specific (abdominal pain, weight loss, malaise and hypotension)
  • Bilateral hydronephrosis and renal failure.
  • Secondary- it is bilateral.
  • Hypertension, lower limb oedema
  • Ureteric obstruction commonly in lower 1/3rd causing dysuria, oliguria and renal failure.
  • Localised or generalised
  • Obstructive uropathy is the earliest and MC specific symptom.
  • The major structure involved are-

     1)  Ureter – Most commonly involved

     2) Aorta

     3) Inferior venacava

 

INVESTIGATIONS-

  • IVP or RGP-

a)      Deviation of middle 1/3rd of ureter medially

b)      Hydroureteronephrosis

c)      Extrinisic ureteral compression

  • CT scan is IOC
  • Raised ESR and CRP

 

TREATMENT-

 

  • Corticosteroids, with or without surgery, are the mainstay of medical therapy.
  1. Primary idiopathic RPF-
  • Ureter stenting and immunosuppression- (TAPS- tamoxifen, azathioprine, penicillamine, steroids)

     2. Secondary RPF-

  • Midline transperitoneal ureterolysis with wrapping the ureter with omental flap or lateral retroperitoneal ureteral transposition.

Exam Important

  • RPF is an uncommon inflammatory condition characteristed by proliferation of fibrous tissue in the retroperitoneum.
  • Group of disordee associated with mediastinal fibrous, Duputyren’s contracture, plantar fasciitis, Peyronie’s disease, Riedel’s thyroiditis, sclerosing cholangitis, mesntric panniculitis, pseudotumour of orbit and fibromatosis.
  • The process usually begins at the level of aortic bifurcation and spreads cephalad upto renal artery.
CLINICAL FEATURES-

  • Patient present with severe and persistent back pain
  • Males of 40- 60 years
  • Early symptoms are vague and non- specific (abdominal pain, weight loss, malaise and hypotension)
  • Bilateral hydronephrosis and renal failure.
  • Secondary- it is bilateral.
  • Hypertension, lower limb oedema
  • Ureteric obstruction commonly in lower 1/3rd causing dysuria, oliguria and renal failure.
  • Localised or generalised
  • Obstructive uropathy is the earliest and MC specific symptom.
  • The major structure involved are?

     1)  Ureter – Most commonly involved

     2) Aorta

     3) Inferior venacava

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