대한흉부영상의학회 Korean Society of Thoracic Radiology GuerBet

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대한흉부영상의학회 Weekly Case 검색
대한흉부영상의학회 Weekly Case 검색
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Weekly Chest CasesArchive of Old Cases

Case No : 25 Date 1998-04-20

  • Courtesy of Choong-Ki Park, M.D. / Hanyang Univercity Kuri Hospital
  • Age/Sex 39 / M
  • Chief Complaintanterior chest pain
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Aortic Dissection (Type A) in Marfan Syndrome
Radiologic Findings
Brief Review
Marfan syndrome is an autosomal dominant disorder with a variable expression involving the eyes ( myopia, ectopia lentis), aorta and heart ( aortic aneurysm, aortic regurgitation, mitral valve disease), and musculoskeletal system ( long limbs, arachnodactyly, pectus deformities, and joint laxity). Recent studies have demonstrated abnormalities of fibrillin caused by mutations of the fibrillin gene on chromosome 15. Life expectancy is greatly reduced, with most deaths caused by cardiovascular complications. The sudden onset of chest pain in a patient with Marfan syndrome may indicate this cardiovascular complication, aortic dissection of the aorta should be considered in the differential diagnosis, particularly during pregnency. The predominant intrathoracic abnormalities are emphysema, scoliosis and cor pulmonale. Other manifestations include bullae, upper lobe fibrosis, frequent respiratory infection, bronchial hyper-reactivity, bronchiectasis and dissecting aneurysm of the pulmonary artery, and pneumothorax.
References
Keywords
Vascular, Vascular,

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