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Imaging of Marfan Syndrome: Multisystemic Manifestations¹

¹ From the Departments of Radiology and the Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 388-1 Pungnap-2 dong, Songpa-gu, Seoul 138-736, Korea. Presented as an education exhibit at the 2005 RSNA Annual Meeting. Received September 18, 2006; revision requested October 24 and received December 18; accepted December 18. All authors have no financial relationships to disclose. Address correspondence to J.B.S. (e-mail: seojb{at}amc.seoul.kr).

Marfan syndrome is an inherited multisystemic connective-tissue disease that is caused by a mutation of the fibrillin-1 gene. The syndrome is characterized by a wide range of clinical manifestations. Common cardiovascular manifestations, most of which are substantial contributors to mortality, include annuloaortic ectasia with or without aortic valve insufficiency, aortic dissection, aortic aneurysm, pulmonary artery dilatation, and mitral valve prolapse. Scoliosis, pectus excavatum and carinatum, arachnodactyly, and acetabular protrusion are common musculoskeletal manifestations. Dural ectasia is a characteristic central nervous system manifestation. In some patients with Marfan syndrome, there is also pulmonary and ocular involvement. Early identification and treatment of these conditions contribute to an improved quality of life and a life expectancy close to the average for the general population in the United States. Radiologists play a key role in the diagnosis of Marfan syndrome. Knowledge about the various manifestations of Marfan syndrome and awareness of their radiologic appearances permit a comprehensive diagnostic approach that allows better patient care.

© RSNA, 2007

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