Volume : VII, Issue : VII, July - 2018

Case of Marfan's syndrome presenting a Spontaneous Pneumothorax

Dr. Raaghav Gupta

Abstract :

 A case of Right sided Spontaneous pneumothorax in a 12 year old girl with previously undiagnosed Marfan’s syndrome. On physical examination

she presented with marfanoid features.SP was managed with Intercostal chest tube drainage and Video assisted thoracic surgery. Marfan
Syndrome itself and its respiratory manifestastions are not common , hence patients who are clinico–etiologically suspicious of spontaneous
pneumothorax, marfan syndrome as a probable etiology should be kept in mind .
Background :–Spontaneous pneumothorax (SP) is a relatively rare condition in paediatric population with a bimodal peak age of occurance either
in neonatal period or in late adolescence. In children SP is often caused by a tear in visceral pleura due to rupture of a subpleural bulla & often affects
tall individuals with thin built1.
Marfan syndrome is a common inherited connective tissue disorder with typical skeletal, ocular and cardiovascular manifestations. Pulmonary
involvement occurs less frequently, with SP being the most frequently reported (4–11%)2–4.
Marfan syndrome should be suspected in patients with SP and marfanoid habitus.

Keywords :

Article: Download PDF   DOI : 10.36106/ijsr  

Cite This Article:

Dr.Raaghav Gupta, Case of Marfan's syndrome presenting a Spontaneous Pneumothorax, INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH : Volume-7 | Issue-7 | July-2018


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