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[Morbus Ormond (idiopatic retroperitoneal fibrosis)]

A Michaligová1, L Plank, A Jezíková

  • 1I. Interná klinika Jesseniovej lekárskej fakulty UK a Univerzitnej nemocnice Martin, Slovenská republika. michaligova@gmail.com

Vnitrni Lekarstvi
|June 24, 2011
PubMed

Insights

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition affecting the aorta. This case report details a 37-year-old male diagnosed with IRF, also known as Morbus Ormond, presenting with fever and inflammation.

Area of Science:

  • Nephrology
  • Vascular Surgery
  • Radiology

Background:

  • Idiopathic retroperitoneal fibrosis (IRF) is a rare fibrotic condition typically affecting the abdominal aorta and iliac arteries, often involving adjacent structures like ureters and the inferior vena cava.
  • IRF commonly presents in individuals aged 40-60, with a male predominance, and frequently manifests as ureteral obstruction.
  • The condition, also known as Morbus Ormond, is characterized by extensive retroperitoneal fibrosis of unknown etiology.

Observation:

  • A 37-year-old male presented with persistent fever (approx. 38°C) and elevated inflammatory markers despite empirical antibiotic treatment.
  • Positron emission tomography (PET) revealed localized, hypermetabolic activity of fluorodeoxyglucose (FDG) predominantly in the left para-aortic region.
  • Microscopic examination of an extracted retroperitoneal mass confirmed the presence of fibrous and inflammatory components.

Findings:

  • The clinical presentation, imaging findings (PET scan), and histological analysis were consistent with a diagnosis of idiopathic retroperitoneal fibrosis.
  • The patient's younger age at onset (37 years) is noteworthy compared to the typical age range for IRF.
  • The para-aortic location of the fibrotic process was clearly identified through PET imaging.

Implications:

  • This case highlights the importance of considering IRF in younger patients presenting with unexplained fever and elevated inflammatory markers, even without classic ureteral compression symptoms.
  • Advanced imaging techniques like PET-CT are crucial for localizing the fibrotic process and aiding in diagnosis.
  • Accurate diagnosis of IRF is essential for appropriate management and to differentiate it from other retroperitoneal masses or inflammatory conditions.

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