Osteo-neuro-meningeal-lumbosacral involvement in Marfan syndrome. Report of a case
Boyer, A.; Marie, I.; Primard, E.; Cailleux, N.; Lévesque, H.; Courtois, H.
Journal des Maladies Vasculaires 24(2): 139-142
1999
ISSN/ISBN: 0398-0499 PMID: 10399648 Document Number: 508881
Marfan syndrome is an hereditary condition which primarily affects conjunctive tissue with predominant vascular lesions, aortic insufficiency and aortic dissection which condition vital prognosis. Until further progress is made in the genetic determination of the disease, the diagnosis is currently based on the association of clinical criteria, which enables multidisciplinary management. This approach should lead to specific medical and surgical treatment--which may reduce Marfan morbidity and mortality. We report the case of a 45 year-old patient with suspected Marfan syndrome during adolescence. The presence of a cardiovascular lesion and a recently reported abnormality i.e. a sacral erosion by a dural ectasia, enabled us to confirm the diagnosis. We reviewed the current criteria in Marfan diagnosis and their specific management.