title: Persistent spontaneous pneumothorax in an adolescent with Marfan's syndrome and pulmonary bullous dysplasia.
abstract: A 16-year-old boy with Marfan's syndrome was admitted with progressive dyspnea due to a large spontaneous pneumothorax. Bullous pulmonary dysplasia was confirmed and pleural tube drainage did not affect the air leak. Complete recovery required surgical resection of the bulla responsible for the ongoing air leak. This case report highlights the issue of management for severe spontaneous pneumothorax in general, showing that the choice of treatment should not depend on the presence of pulmonary bullous dysplasia but on the clinical evaluation of the individual patient.
authors.affiliation: Department of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy. iclpe@rm.unicatt.it
authors.forename: D
authors.initials: D
authors.lastname: Rigante
authors.forename: G
authors.initials: G
authors.lastname: Segni
authors.forename: A
authors.initials: A
authors.lastname: Bush
mesh_terms: D000293:Adolescent; D001768:Blister / Q000150:complications*; D006801:Humans; D008171:Lung Diseases / Q000150:complications*; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D011030:Pneumothorax / Q000209:etiology* / Q000601:surgery