Pneumothorax and Bilateral Honeycombed Lung in Marfan Syndrome

Abstract

Pneumothorax occurred in a 17-year-old boy with previously undiagnosed Marfan syndrome. Radiographic evidence of interstitial lung disease with honeycomb changes in the upper thirds of both lungs was present. Pulmonary function tests revealed only slight decrease in lung volumes and expiratory flows possibly resulting from large predicted normal values because of the unusual height characteristic of this disorder. The lung expanded without difficulty and pneumothorax did not recur. The occurrence of pneumothorax and parenchymal lung disease in this syndrome might reflect a localized pulmonary abnormality related to the generalized connective tissue defect.