Spontaneous Pneumothorax and Pulmonary Disease in the Marfan Syndrome

doi:10.1059/0003-4819-62-6-1285

Spontaneous Pneumothorax and Pulmonary Disease in the Marfan Syndrome

Report of Two Cases and Review of the Literature

EDWARD M. DWYER, JR., M.D.; and
FRANK TRONCALE, M.D.

Requests for reprints should be addressed to Edward M. Dwyer, Jr., M.D., Cardiovascular Laboratory, Presbyterian Hospital,
622 W. 168th Street, New York, N. Y. 10032
.

Excerpt

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

Acknowledgment

The constructive criticisms of Drs. Theodore B. Van Itallie and A. L. Loomis Bell, Jr., are gratefully acknowledged.

Summario in Interlingua

Es reportate duo casos de pneumothorace spontanee associate con le syndrome de Marfan. Esseva constatate que altere lesiones pulmonari incontrate frequentemente in association con le syndrome de Marfan include malformationes congenite del pulmon, emphysema pulmonari, e morbo cystic del pulmon. Le pathogenese e le pathologia de iste lesiones es discutite. Anormalitate del function pulmonari ha previemente essite describite. In ambe nostre casos un declino del saturation a oxygeno esseva constatate post exercitio. Es signalate le urgente desiderato de un plus complete evalutation del stato pulmonari de patientes con le syndrome de Marfan.