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• W2067359685 abstract "Le syndrome de Marfan est une maladie génétique rare liée à la mutation du gène FBN1, codant la fibrilline. Les atteintes cardiovasculaires, squelettiques et ophtalmologiques sont les plus fréquentes, mais la maladie peut toucher le poumon. Les pneumothorax atteignent 5 à 11 % des patients. Le rôle favorisant des déformations thoraciques (pectus excavatum et carinatum) ou des blebs reste débattu. Leur prise en charge est identique à celle de tout pneumothorax spontané, mais le risque de récidive est élevé. Le pectus excavatum touche jusqu’à 60 % des patients, le plus souvent sans retentissement fonctionnel. En cas de mauvaise tolérance, un traitement chirurgical peut être envisagé par la technique de Nuss. La prévalence du syndrome d’apnées obstructives du sommeil semble élevée dans le syndrome de Marfan, et sa présence pourrait contribuer à la dilatation aortique. La responsabilité des anomalies cranio-faciales associées aux apnées du sommeil a été évoquée, sans certitude à ce jour. Il est important pour un pneumologue d’évoquer le diagnostic de syndrome de Marfan chez un patient présentant des pneumothorax spontanés récidivants ou une déformation thoracique, car la prise en charge précoce de l’atteinte cardiaque conditionne le pronostic. Marfan's syndrome is a rare genetic disorder caused by a mutation of the gene FBN1, coding for the protein fibrillin-1. Cardiovascular, musculoskeletal and ophthalmic manifestations are the most commonly observed, but minor diagnostic criteria also include pulmonary manifestations. Pneumothorax, frequently relapsing, affects 5 to 11% of patients. Rib cage abnormalities (pectus excavatum or pectus carinatum) and apical blebs may contribute to their occurrence. Treatment does not require any specific procedure but there is an increased risk of recurrence. Pectus excavatum affects up to 60% of the patients, without any functional impairment in most cases. Surgery may be required (using the Nuss procedure) in case of cardiovascular or psychological symptoms. Marfan's syndrome is frequently associated with obstructive sleep apnoea, which may itself contribute to aortic dilatation. Some studies suggest a potential role of craniofacial abnormalities in the pathogenesis of sleep apnea in these patients. Pulmonologists should consider Marfan's syndrome when treating patients for recurrent spontaneous pneumothorax or rib cage abnormalities, since early detection of cardiac abnormalities improves the prognosis significantly." @default.
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• W2067359685 date "2015-02-01" @default.
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• W2067359685 title "Manifestations respiratoires du syndrome de Marfan" @default.
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• W2067359685 doi "https://doi.org/10.1016/j.rmr.2014.06.030" @default.
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