HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): John R. Hall Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Hall, J. R. Articles by Haller, J. A. Search for Related Content PubMed PubMed Citation Articles by Hall, J. R. Articles by Haller, J. A., Jr.

Ann Thorac Surg 1984;37:500-504
© 1984 The Society of Thoracic Surgeons

---

Articles

Pneumothorax in the Marfan Syndrome: Prevalence and Therapy

From the Departments of Surgery, Medicine, and Pediatrics, the Johns Hopkins University School of Medicine, Baltimore, MD

Accepted for publication November 10, 1983.

^* Address reprint requests to Dr. Pyeritz, Moore Clinic, Johns Hopkins Hospital, Baltimore, MD 21205

At least twenty-four reports of pneumothorax associated with the Marfan syndrome have appeared in the medical literature, but the frequency and optimal method of treatment remain unclear. We conducted a retrospective review of medical records from one genetics clinic and found that the frequency of spontaneous pneumothorax in patients older than 12 years with Marfan's syndrome was 4.4% (11 out of 249). Men were more commonly, but no more severely, affected than women. Seven patients had recurrent or bilateral pneumothorax. In 9 of the 11 patients, apical bullae were present and detectable on chest radiograph. Pneumothorax frequently recurred if not treated by resection of the offending bullae. We recommend that all adolescent and adult patients with the Marfan syndrome undergo chest radiography for ascertainment of the gross pathological condition of the lung. Definitive surgical treatment should be performed at the first occurrence of pneumothorax because of the high rate of recurrence after treatment with a chest tube, with or without pleurodesis.

This article has been cited by other articles:

				K. Dyhdalo and C. Farver Pulmonary Histologic Changes in Marfan Syndrome: A Case Series and Literature Review Am J Clin Pathol, December 1, 2011; 136(6): 857 - 863. [Abstract] [Full Text] [PDF]

				L. E. D. Shirley and P. D. Sponseller Marfan Syndrome J. Am. Acad. Orthop. Surg., September 1, 2009; 17(9): 572 - 581. [Abstract] [Full Text] [PDF]

				J. R. Toro, S. E. Pautler, L. Stewart, G. M. Glenn, M. Weinreich, O. Toure, M.-H. Wei, L. S. Schmidt, L. Davis, B. Zbar, et al. Lung Cysts, Spontaneous Pneumothorax, and Genetic Associations in 89 Families with Birt-Hogg-Dube Syndrome Am. J. Respir. Crit. Care Med., May 15, 2007; 175(10): 1044 - 1053. [Abstract] [Full Text] [PDF]

				C.-C. Lee, S.-H. Lee, I.-J. Chang, and W.-J. Chen Spontaneous pneumothorax are seen in the joint hypermobility syndrome (JHS): reply Rheumatology, August 1, 2006; 45(8): 1052 - 1053. [Full Text] [PDF]

				B. J. Maron, B. R. Chaitman, M. J. Ackerman, A. Bayes de Luna, D. Corrado, J. E. Crosson, B. J. Deal, D. J. Driscoll, N.A. M. Estes III, C. G. S. Araujo, et al. Recommendations for Physical Activity and Recreational Sports Participation for Young Patients With Genetic Cardiovascular Diseases Circulation, June 8, 2004; 109(22): 2807 - 2816. [Abstract] [Full Text] [PDF]

				R. Kanno, H. Suzuki, K. Fujiu, Y. Yoshino, A. Ohishi, and M. Gotoh Hemopneumothorax associated with marfan syndrome and congenital afibrinogenemia Ann. Thorac. Surg., April 1, 2003; 75(4): 1304 - 1306. [Abstract] [Full Text] [PDF]

				M. Cohen and S. A. Sahn Bronchiectasis in Systemic Diseases Chest, October 1, 1999; 116(4): 1063 - 1074. [Full Text] [PDF]

				R. E. PYERITZ Connective Tissue in the Lung: Lessons from the Marfan Syndrome Ann Intern Med, August 1, 1985; 103(2): 289 - 290. [Abstract] [PDF]