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Retroperitoneal fibrosis in Marfan's syndrome

W K Chong1, M A al-Kutoubi

  • 1Department of Radiology, St Mary's Hospital, London.

Clinical Radiology
|December 1, 1991
PubMed

Insights

This study identifies a novel association between Marfan

Area of Science:

  • Cardiovascular Medicine
  • Nephrology
  • Radiology

Background:

  • Marfan's syndrome is a genetic disorder affecting connective tissue, primarily known for its cardiovascular manifestations.
  • Retroperitoneal fibrosis (RPF) is a condition characterized by inflammatory tissue in the retroperitoneum, often leading to urinary tract obstruction.

Observation:

  • Two patients with Marfan's syndrome presented with acute aortic disease.
  • Both patients were subsequently diagnosed with undiagnosed retroperitoneal fibrosis (RPF) causing hydronephrosis.
  • Computed tomography (CT) revealed these findings, demonstrating hydronephrosis secondary to RPF.

Findings:

  • This report describes the first known association between Marfan's syndrome and retroperitoneal fibrosis (RPF).
  • The co-occurrence is biologically plausible given current hypotheses on the pathogenesis of aortic disease in Marfan's syndrome and the etiology of RPF.
  • CT imaging plays a crucial role in diagnosing RPF and assessing aortic complications in Marfan's syndrome.

Implications:

  • Increased awareness of RPF in Marfan's syndrome patients may facilitate earlier diagnosis.
  • Early detection and management of RPF can prevent significant renal impairment in affected individuals.
  • This association highlights the importance of comprehensive evaluation in patients with Marfan's syndrome.

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