This document discusses a case of retroperitoneal fibrosis in a 42-year-old female patient. It provides details of her medical history and treatments over the past 2 years for her condition. Imaging showed retroperitoneal fibrosis encasing the right ureter. She underwent right ureterolysis with uretero-ureterostomy and omental wrapping. Post-operatively, her drain output was serous and she was discharged after ensuring no collections were present. Retroperitoneal fibrosis is defined as an inflammatory fibrotic process in the retroperitoneum commonly causing ureteral compression. It most often presents with back or flank pain and can be idiopathic or associated with various causes. Management involves

1. RETROPERITONEAL FIBROSIS
(Case Discussion)
Dr Shuah Ullah
Resident

2. Case History :
Patient Najma, 42 years old female, Known case of HTN, married,
resident of Shikarpur, diagnosed case of retroperitoneal fibrosis for last
2 years presented to SIUT in August 2018 for better management.
On presentation she has complain of B/L Flank pain.

3. She has history of current disease started from January 2017.
Took steroids for almost one year.
B/L DJs replaced 3 times in last one and half years outside SIUT.

11. CT Abdomen & Pelvis Feb. 2018

17. CT Abdomen & Pelvis July 2018

24. Investigations at SIUT :
Blood CP : HB 9.7mg/dl S. Cre 1.16
PCV 31.1
TLC 5.5
PLT 286000
INR 1.01

25. Urine DR : RBC & Pus cells Numerous.
Urine CS : No Growth
ULTRASOUND :
Right : 10.2 cm, Mild residual HDN, prominent ureter, DJs in situ.
Normal texture
Left : Small 6.4 cm echogenic texture, loss of cortex, no HDN/HU,
No Calculi. DJs in situ.
Urinary Bladder: Distal loop of DJs, partially filled.

26. Right Ureterolysis + Uretero-ureterostomy + Omental Wrapping + Right
DJs Replacement was done on 24-09-2018.

27. Intra operative Findings :
Large area of retroperitoneal fibrosis encasing major vessels, proximal and mid
ureter on right side.
Right Kidney was healthy.
Right ureter adhesion lysis was done and was mobilized along its entire length.
Ureter 1-1.5 cm necrotic segment sacrificed.
End to end ureteric anastomosis done with healthier segments of ureter after
replacing DJs.
Omentum wrapped circumferentially around proximal and mid ureter.

28. Follow Up :
Patient remain admitted for 6 days due to working drain.
Drain biochemistry reveals serous fluid, so drain was removed and pt.
was discharged next day after ensuring no any collection.
Last week she visited SIUT Sukkur where labs and U/S were normal.
Advised for Follow up at opd # 14 on 22-10-2018 for B/L DJs removal.

29. RETROPERITONEAL FIBROSIS :
Inflammatory fibrotic process in retroperitoneum causing compression
of retroperitoneal structures, most commonly ureters.
Most commonly 40-60 years of age.
Male predominance (2:1 to 3:1).

30. Idiopathic in 70% of cases. (Pipitone et al, 2012)
Idiopathic RPF is considered part of the spectrum of chronic peri-
aortitis, a large vessel vasculitis.
Ceriod, polymer of lipid and protein found in atherosclerotic plaques
suggested as an antigen initiating the inflammatory response.

31. Rest 30% has an identifiable cause including.
DRUGS : Methysergide (Sansert) and other ergot alkaloids, Beta
blockers, Phenacetin.
MALIGNANCY : Lymphoma, Multiple Myeloma, Carcinoid, Pancreas,
Prostate, Sarcoma.
RADIOTHERAPY
ASBESTOS Exposure via G.I and Pulmonary lymphatic drainage.
INFECTIONS : TB, Actinomyces, Gonorrhea, Schistosomiasis.

32. Most commonly presenting symptoms is pain in lower back or flank,
dull, non colicky, unchanged with posture.
Other symptoms include, weight loss, anorexia, nausea, generalized
malaise, fever, hypertension, oliguria or anuria, DVT and lower
extremity edema (IVC compression), Renal vein hypertension and gross
hematuria (R.V Compression)

33. Investigations :
• Raised Urea and Creatinine (50-75 %)
• Inflammatory markers like CRP and ESR. Raised in one half to two
thirds of patients.
• ANA (60 % cases)
• Anemia and leukocytosis.

34. Imaging :
CT Urogram or Xray IVP : Dilated PCS with medial deviation of proximal and mid ureter
which smoothly tapered at the level of obstruction( usually L4-L5).
MRI : T2 high signals with active disease.
PET : Most sensitive for disease activity.
Nuclear Imaging
Retrograde Pyelography

35. Biopsy
• Percutaneous (Tru-Cut), U/S or C.T guided.
• Laparoscopic (preferable).
• At time of ureterolysis (open or laparoscopic)

36. Medical Management :
• Steroid therapy
80 % clinical response including dec. in size of mass and improvement
in compression symptoms.
Initial dose 60 mg/day tapered to 5 mg/day.
25-50% relapses occurs during tapering.

37. • Tamoxifen
Early response time and low adverse effects profile than steroids.
Low remission rates, higher relapse rates doesn’t makes it suitable as
monotherapy.
0.5 mg/kg/day
• Immunosuppressive agents
Azathioprine, Cyclophosphamide, cyclosporine, Mycophenolate mofetil etc.

38. Surgical Management :
• Temporary measures like PCN and DJs.
• Ureterolysis (Lap or Open) with omental wrapping of ureter.
• Renal Auto-transplantation.

39. Ureterolysis :
• Lap. Reported as shorter hospital stay (open 5.9 vs lap 2.1 days).
Complication rates remain same.
Success rate, open 87.5% vs Lapro 93.8 % ( Steyn et al, 2011)

41. Proposed therapeutic algorithm for
idiopathic RPF. IS,
immunosuppressants (particularly
mycophenolate mofetil, methotrexate,
azathioprine, cyclophosphamide); RTX,
rituximab; TCZ, tocilizumab.
Augusto Vaglio and Federica Maritati
JASN July 2016, 27 (7) 1880-1889; DOI:
https://doi.org/10.1681/ASN.2015101110

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