retroperitoneale Fibrose

US
confirmation of hepatopetal flow through bladder-umbilical-portal pathway in a patient affected by inferior vena cava obstruction due to retroperitoneal fibrosis and pelvic lipomatosis. CTI (a/b) and T2WI MRI (c) visualize soft tissue which spares the great vessels, is mainly located distal to the renal hilum and has an infiltrative aspect enveloping rather than displacing adjacent structures.
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Retroperitoneal
fibrosis + double inferior cava vein + left kidney agenesis. Soft-tissue mass circumferentially surrounding the aorta. There is absent contrast enhancement which suggests no active inflammation.
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Retroperitoneal
fibrosis + double inferior cava vein + left kidney agenesis. Mass around the aorta that has a signal intensity similar to muscle. There is no anterior displacement of the aorta.
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Retroperitoneal
fibrosis + double inferior cava vein + left kidney agenesis. Retroperitoneal mass surrounding the aortic artery. The low signal intensity of this tissue suggests no active inflammation.
www.eurorad.org • CC by-nc-sa-4.0
Retroperitoneal
fibrosis + double inferior cava vein + left kidney agenesis. Low-attenuation mass anterior and lateral to the aorta. The mass does not cause anterior aortic displacement. Double inferior cava vein and left kidney agenesis are also seen. There is no hydronephrosis or hydroureter.
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Retroperitoneal
fibrosis + double inferior cava vein + left kidney agenesis. Transverse and longitudinal ultrasound at level of mid aorta reveals the presence of a preaortic and paraaortic hypoechoic soft tissue mass. The aortic artery has a normal size.
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Retroperitoneal
fibrosis • Retroperitoneal fibrosis (autoimmune) post bilateral ureterolysis - Ganzer Fall bei Radiopaedia
O'Donnell C, Retroperitoneal fibrosis (autoimmune) post bilateral ureterolysis. Case study, Radiopaedia.org (Accessed on 15 Aug 2023) https://doi.org/10.53347/rID-37871 • CC by-nc-sa 3.0 (modified)
US
confirmation of hepatopetal flow through bladder-umbilical-portal pathway in a patient affected by inferior vena cava obstruction due to retroperitoneal fibrosis and pelvic lipomatosis. CTI (a/b) and T2WI MRI (c) visualize soft tissue which spares the great vessels, is mainly located distal to the renal hilum and has an infiltrative aspect enveloping rather than displacing adjacent structures.
www.eurorad.org • CC by-nc-sa-4.0
A case of
idiopathic retroperitoneal fibrosis presenting as gallbladder carcinoma. Characteristics of the abdominal CT scan. Abdominal computed tomography scan showing the gallbladder occupying and involving the hilar bile duct and dilatation of the proximal biliary system (a); posterior peritoneal mass occupying/involving the upper part of the bilateral ureter (b), and the change in the proximal urinary tract (c)
bmcsurg.biomedcentral.com • CC-by-4.0
Use of
rituximab in idiopathic retroperitoneal fibrosis. CT scan of Patient 2 pre-treatment (a) demonstrating a soft tissue mass (arrow) in keeping with RPF partially encasing the aorta, IVC and the right ureter. CT scan performed subsequently in the same patient post treatment (b) demonstrates that the soft tissue representing RPF has almost completely resolved. There is only a small amount of residual tissue (arrow) surrounding the right ureter, which has a stent in place
bmcrheumatol.biomedcentral.com • CC-by-4.0
US
confirmation of hepatopetal flow through bladder-umbilical-portal pathway in a patient affected by inferior vena cava obstruction due to retroperitoneal fibrosis and pelvic lipomatosis. MRI shows low T2 signal intensity reflecting poor or absent inflammation activity due to glucocorticosteroid therapy.
www.eurorad.org • CC by-nc-sa-4.0
Retroperitoneal
fibrosis + double inferior cava vein + left kidney agenesis. Absence soft-tissue enhancement after contrast administration, which indicates inactive disease.
www.eurorad.org • CC by-nc-sa-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Retroperitoneal fibrosis (RPF), is a condition that has previously been described as chronic periaortitis. It is an uncommon fibrotic reaction in the retroperitoneum that typically presents with ureteric obstruction.

The disease is part of a spectrum of entities that have a common pathogenic process consisting of an inflammatory response to advanced atherosclerosis of the abdominal aorta, combined with autoimmunologic factors:

Epidemiology

Retroperitoneal fibrosis is an uncommon condition with a reported estimated incidence of 1.38 cases per 100,000 people . The mean age is approximately 64 years with a male-to-female ratio of 3:1 .

Clinical Presentation 

They can present with systemic symptoms such as mild fever, malaise due to an ongoing inflammatory process. Some times they get flank or back pain which is not position-dependent and has a transient relief with oral non-steroidal anti-inflammatory drugs . If the ureters are involved, may develop a colicky ureteric pain.

Pathology

Etiology

Radiographic features

General principles

Contrast-enhancing fibrosis encasing the retroperitoneal structures causing ureteric and vascular obstruction and displacement.

Displacement of the aorta and IVC anteriorly away from the vertebral bodies are suggestive of malignant etiology. Invasion and disruption of bone and/or soft tissue structures suggests an aggressive process - infection or malignancy.  Multiple subcentimeter lymph nodes are frequently seen in non-malignant RPF, probably reactive secondary to the disease process, and should not be confused for malignancy .

Fluoroscopy: IVU

While largely superseded by cross-sectional imaging techniques, a classic triad is described :

  • medial deviation of the middle third of the ureters
  • tapering of the lumen of one or both ureters in the lower lumbar or upper sacral region
  • proximal unilateral or bilateral hydroureteronephrosis with delayed excretion of contrast material (reported in over half of cases and thought to be due to impaired peristalsis)
    • CT

    Retroperitoneal fibrosis is visible as a soft tissue density mass located around the aorta and iliac arteries. Classically, it develops around the aortic bifurcation and spreads upwards where it can envelop the renal hila. It encases but does not invade or stenose the ureters or vessels. However, ureteric obstruction and venous thromboses can occur.

    In early or active stages, variable enhancement can be seen with intravenous contrast while no enhancement may be seen in the quiescent disease.

    MRI

    MRI has been reported to be as sensitive as CT in its assessment of retroperitoneal fibrosis with the added advantage of high contrast resolution between closely apposed retroperitoneal structures. It can evaluate the urinary tracts using fast T2 weighted spin-echo sequences without requiring intravenous contrast in patients with impaired renal function.

    The soft tissue mass is usually dark on T1W and T2W unless there is an active inflammation whereby the T2W images can be hyperintense.

    FDG-PET

    Uptake will be seen in active inflammation and absent in metabolically inactive disease .

    Treatment and prognosis

    Retroperitoneal fibrosis may lead to ureteric obstruction and consequent renal failure. In some cases, a blow-out of the ureter is described as a result of the obstruction.

    Differential diagnosis

    Imaging differential considerations include:

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