Further Outpatient Care

Biochemical markers (eg, C-reactive protein, erythrocyte sedimentation rate, renal function) should be monitored every 4-8 weeks to assess the response.

Radiologic assessment (eg, CT scanning, MRI) is performed every 3 months. Once disease is stabilized, scanning can be repeated at 6 months.

Recurrence of stenosis has been reported as late as 10 years; thus, long-term follow-up is necessary.

Patients with renal failure should be referred to a nephrologist early in the course of their disease and have continued nephrologic follow-up. Renal recovery is usually observed within the first 2 weeks, but some patients may not regain renal function until much later.

Further Inpatient Care

After relief of long-standing obstruction, a physiologic diuresis is expected. This is usually a self-limiting process and can be managed conservatively with fluid and electrolyte replacement.

Urea diuresis is the most common. It is self-limiting, lasting 24-48 hours. Monitoring of fluid balance and electrolytes is required. Unless otherwise contraindicated, increased oral fluid intake should suffice.

Sodium diuresis is the second most common postobstructive diuresis. It usually is self-limiting but may have a longer duration (>72 h). Monitor fluid balance and electrolytes more aggressively (ie, intake and output [I/O], central venous pressure [CVP], urine and serum electrolytes).

Ultrasonography may be used to assess hydronephrosis.

Complications

See the list below:

Renal failure
Hypertension
Scrotal edema
Inferior vena cava thrombosis

Prognosis

The natural history of retroperitoneal fibrosis (RPF) has not been clearly established. However, the outcome of nonmalignant retroperitoneal fibrosis is generally good.^{[72, 73, 74, 75, 76, 77, 78, 79]}Renal recovery is usually observed within the first 2 weeks. Checking these patients periodically is always better because some patients may regain renal function much later. The prognosis of malignant retroperitoneal fibrosis is poor.

Patient Education

Patients with renal failure should be educated about the following:

The importance of compliance with secondary preventative measures
Natural disease progression
Prescribed medications (highlighting their potential benefits and adverse effects)
Diet

Questions

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Media Gallery

Intravenous urogram shows medial deviation of the middle part of both ureters.
Retrograde ureterogram reveals smooth narrowing and medial shift of the ureter.
Retrograde pyelogram demonstrates hydronephrosis.
Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.
Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.
Management algorithm of retroperitoneal fibrosis.
Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.
Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.
Abdominal radiograph demonstrates a wall stent on the right side.

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Table. Differential Diagnoses of Retroperitoneal Fibrosis^[43]

Table. Differential Diagnoses of Retroperitoneal Fibrosis ^[43]

	Retroperitoneal Fibrosis	Retroperitoneal Lymphoma	Sclerosing Mesenteritis	Desmoid-Type Fibromatosis	Inflammatory Myofibroblastic Tumor	Well-Differentiated Liposarcoma Sclerosing Variant
Ureteral displacement	Medial	Lateral
Ureteral obstruction	~80%	~50%	Rare	Rare	Rare	Unknown
Aortic displacement	Rare	Anterior
Reactive perivascular lymphoid aggregates	100%	Absent	Variable	Rare	Variable	Present in the inflammatory type
Necrosis	Absent	Variable	Fat necrosis	Rare	Focal	Fat necrosis
Vasculitis	~50%	Absent	Absent	Absent	Absent	Absent
Clonality	Absent	Variable	Absent	Absent	Absent	Present
Β-catenin	Negative	Unknown	Negative	Positive in 90% of cases	Negative	Variable positivity
ALK-1	Negative	Usually negative	Negative	Negative	Positive in 50% of cases	Negative
CD-117	Negative in spindle cell component	Rare	Variable	Negative	Rare	Negative
Desmin	Negative	Negative	Variable	Rare	Usually positive	Rare
S100	Negative	Negative	Negative	Rare	Negative	Usually positive in the adipocytic component

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Author

Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU Consulting Urologist, Department of Urology, Pinderfields General Hospital, The Mid-Yorkshire Hospitals NHS Trust, UK

Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU is a member of the following medical societies: British Medical Association, International College of Surgeons, British Association of Urological Surgeons, European Association of Urology

Disclosure: Nothing to disclose.

Coauthor(s)

Joby Taylor, MBChB, FRCS Consultant Urologist, Forth Valley Royal Hospital, UK

Joby Taylor, MBChB, FRCS is a member of the following medical societies: British Association of Urological Surgeons, European Association of Urology, International Continence Society, Royal College of Surgeons of Edinburgh, United Kingdom Continence Society

Disclosure: Nothing to disclose.

Anthony J Browning, MB, ChB, FRCS(Edin) Consultant Urological Surgeon, Mid Yorkshire NHS Trust, Pinderfields General Hospital; Associate Post Graduate Dean, Yorkshire and Humber Deanery, UK

Anthony J Browning, MB, ChB, FRCS(Edin) is a member of the following medical societies: Endourological Society, British Association of Urological Surgeons, European Association of Urology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Bradley Fields Schwartz, DO, FACS Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, Society of University Urologists

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Endourological Society Board of Directors; President Elect North Central Section of the American Urological Association<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cook Medical.

Additional Contributors

Martha K Terris, MD, FACS Professor and Chief of Urology, Witherington Distinguished Chair, Department of Surgery, Section of Urology, Director, Urology Residency Training Program, Medical College of Georgia at Augusta University; Professor, Department of Physician Assistants, Medical College of Georgia School of Allied Health; Chief, Section of Urology, Augusta Veterans Affairs Medical Center

Martha K Terris, MD, FACS is a member of the following medical societies: American Cancer Society, American College of Surgeons, American Institute of Ultrasound in Medicine, American Society of Clinical Oncology, American Urological Association, Association of Women Surgeons, New York Academy of Sciences, Society of Government Service Urologists, Society of University Urologists, Society of Urology Chairpersons and Program Directors, Society of Women in Urology

Disclosure: Nothing to disclose.