UROLOGY · CONDITIONS
Retroperitoneal Fibrosis
Fibrous tissue encasing the ureters and retroperitoneal structures causing bilateral ureteral obstruction , requires immunosuppression and surgical ureterolysis.
ABOUT THIS CONDITION
What is Retroperitoneal Fibrosis?
Retroperitoneal fibrosis (RPF) is a rare condition in which fibrous tissue forms around the abdominal aorta and inferior vena cava, progressively encasing and compressing the ureters. Most cases are idiopathic , likely immune-mediated , though some are secondary to drugs, malignancy, or radiation. Bilateral ureteral obstruction leads to renal failure if untreated. Dr. Vipin provides emergency decompression with JJ stenting or nephrostomy, biopsy to exclude malignancy, and then definitive ureterolysis with omental wrapping , coordinated with nephrology and rheumatology for concurrent immunosuppressive management.
SIGNS TO WATCH
Common Symptoms
Symptoms that need attention
WHY IT HAPPENS
Causes & Risk Factors
- Idiopathic IgG4-related disease , most common cause (60–70%)
- Methysergide or ergotamine medication exposure
- Aortic aneurysm and periaortic inflammation
- Malignancy , lymphoma, carcinoid, retroperitoneal sarcoma
- Radiation therapy to the retroperitoneum
- Inflammatory bowel disease and autoimmune conditions
CLINICAL DETAILS
Key Facts
IgG4-related disease is now recognised as the most common cause , serum IgG4 and biopsy confirm.
CT-guided biopsy mandatory to exclude malignancy before starting immunosuppression
High-dose corticosteroids induce remission in most idiopathic RPF , long-term low-dose maintenance.
JJ stenting provides immediate ureteral decompression while medical treatment takes effect.
Surgical ureterolysis frees the ureters from the fibrous mass , ureters wrapped in omentum to prevent re-encasement.
30–40% relapse rate on stopping steroids , long-term monitoring of PET-CT activity and creatinine required.
HOW WE TREAT IT
Treatment Approach
Ureterolysis , Open / Laparoscopic
The ureters are surgically freed from the surrounding retroperitoneal fibrous tissue and repositioned laterally or intraperitoneally , the definitive surgical treatment for RPF-related ureteral obstruction.
- 1
Decompression
Bilateral JJ stents placed urgently cystoscopically to relieve ureteral obstruction and protect renal function while diagnosis is confirmed.
- 2
Biopsy & Diagnosis
CT-guided biopsy of the retroperitoneal mass excludes malignancy and confirms fibrous histopathology , IgG4 staining and PET-CT performed.
- 3
Immunosuppression
High-dose oral corticosteroids started , monitored with PET-CT for metabolic response. Tamoxifen or azathioprine added for maintenance.
- 4
Ureterolysis
Laparoscopic or open ureterolysis dissects the ureters free from the fibrous mass. Ureters wrapped in omental flap to prevent re-encasement.
AVAILABLE TREATMENTS
Treatment Options
Ureterolysis , Open / Laparoscopic
Surgical dissection of the ureters from the retroperitoneal fibrous mass , definitive release of ureteral obstruction.
Omental Wrapping of Ureters
Pedicled omental flap wrapped around the freed ureters to prevent re-encasement by fibrous tissue after ureterolysis.
DJ / JJ Stenting
Bilateral internal ureteric stents for immediate decompression while medical immunosuppressive therapy takes effect.
Percutaneous Nephrostomy
Emergency antegrade drainage for severe bilateral obstruction with impending or established acute renal failure.
COMMON QUESTIONS
Frequently Asked
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