Abstract
Background Retroperitoneal fibrosis is a rare disease with an incidence of 0–1/100 000 inhabitants
per year and is associated with chronic inflammatory fibrosis of the retroperitoneum
and the abdominal aorta. This article sheds light on the role of radiological imaging
in retroperitoneal fibrosis, names various differential diagnoses and provides an
overview of drug and surgical treatment options.
Methods A literature search for the keywords “retroperitoneal fibrosis” and “Ormond’s disease”
was carried out in the PubMed database between January 1, 1995 and December 31, 2019
(n = 1806). Mainly original papers were selected, but also reviews, in English and
German language, with a focus on publications in the last 10 years, without excluding
older publications that the authors believe are relevant to the topic discussed in
the review (n = 40).
Results and Conclusion Ormond’s disease is a rare but important differential diagnosis for nonspecific back
and flank pain. Imaging diagnostics using CT or MRI show a retroperitoneal mass, which
must be differentiated from lymphoma, sarcoma, multiple myeloma and Erdheim-Chester
disease. Patients have an excellent prognosis under adequate therapy. FDG-PET/CT or
FDG-PET/MRT should be considered as potential modalities, as hybrid imaging can evaluate
both the morphological changes and the inflammation.
Key Points:
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Ormond’s disease is a differential diagnosis for nonspecific back and flank pain.
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Radiological imaging is essential and the gold standard in the diagnosis and follow-up
of RPF.
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Patients have an excellent prognosis under adequate therapy.
Citation Format
Key words
retroperitoneum - urinary - fibrosis, cystic - inflammation
