Learning objectives
The purpose of this article is to review the imaging features of Idiopathic Retroperitoneal Fibrosis and its differential diagnosis,
illustrated by clinical cases from our practice.
Background
Retroperitoneal fibrosis (RPF) comprises a range of diseases characterized by the proliferation of aberrant fibroinflammatory tissue in the retroperitoneum.
The idiopathic form accounts for more than two thirds of the cases,
with the remaining third being secondary to other factors such as neoplasms,
drugs,
infection,
retroperitoneal haemorrhage,
radiation therapy and proliferative diseases.
Idiopathic Retroperitoneal Fibrosis (also known asOrmond's disease) isa rare condition,
with a prevalence of 1.3 per 100000 population.
Traditionally,it was considered an excessive local inflammatory response to the antigens expressed on atherosclerotic plaques...
Findings and procedure details
Ultrasonography,
although having poor overall sensitivity in the detection of Retroperitoneal Fibrosis,
is usually the first-line study performed to assess the presence of hydronephrosis or associated aortic aneurysm.
A well-defined hypoechoic or isoechoic band anterior to the aorta,
IVC and iliac vesselsmaybe depicted,
sometimes misinterpreted as a retroperitoneal haematoma,
lymphadenopathies or solid neoplasm [Fig.
1].
Computed tomography (CT) is the pillar of noninvasive diagnosis of Idiopathic RPF,
as it allows evaluation ofits location,
extent and effect on adjacent organs and vascular structures.
The typical findings...
Conclusion
The understanding of the typical imaging findings of Idiopathic Retroperitoneal Fibrosis as well as the features that should prompt suspicion for a non-idiopathic aetiology are essential to correctly estabilish the diagnosis and perform adequate treatment.
References
Oliveira R,
Sierra A,
Salvador F,
et al.
Retroperitoneal Fibrosis: Role of Imaging in Diagnosis and Follow-up.
RadioGraphics 2013; 33(2):535-552
Urban ML,
Palmisano A,
Nicastro M,
et al.
Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach.
Rev Med Interne 2015; 36(1):15-21
Cronin C,
Lohan D,
Blake M,
et al.
Retroperitoneal Fibrosis: A Review of Clinical Features and Imaging Findings.
AJR 2008; 191:423-431
Prabhakar R,
Sinha R,
Cuevas C,
et al.
Imaging of Uncommon Retroperitoneal Masses.
RadioGraphics 2011; 31:949-976
Nishino M,
Hayakawa K,
Minami...