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Retroperitoneal fibrosis in Marfan's syndrome
1Department of Radiology, St Mary's Hospital, London.
Clinical Radiology
|December 1, 1991
Summary
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.