Spontaneous Pneumothorax and Pulmonary Disease in the Marfan Syndrome

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Abstract
The Marfan syndrome is a rare but well-defined hereditary disorder of connective tissue (1). Prominent features of the syndrome include abnormalities of the skeletal, cardiovascular, and ocular systems that are manifested as either a congenital anomaly (microphakia, coarctation of the aorta) or a degenerative lesion of elastic tissue (aortic dissection, ectopic lens, hernia). Lesser known areas of involvement are renal (2, 3), dermatologic (4), and pulmonary. The spectrum of pulmonary lesions in the Marfan syndrome includes congenital malformations, cystic disease, emphysema, spontaneous pneumothorax, and susceptibility to respiratory infections. In spite of several recent reports (5-7), the etiology, pathogenesis, and pathology