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Pediatric Reports
Volume 5
Issue 3
10.4081/pr.2013.e15
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Review

Pectus Excavatum and Heritable Disorders of the Connective Tissue

by
Francesca Tocchioni
1,*,
Marco Ghionzoli
1,
Antonio Messineo
1 and
Paolo Romagnoli
2
1
Department of Pediatric Surgery, Children’s Hospital A. Meyer, Florence
2
Department of Anatomy, Histology and Forensic Medicine, University of Florence, Florence, Italy
*
Author to whom correspondence should be addressed.
Pediatr. Rep. 2013, 5(3), e15; https://doi.org/10.4081/pr.2013.e15
Submission received: 14 May 2013 / Revised: 14 May 2013 / Accepted: 13 August 2013 / Published: 24 September 2013

Abstract

Pectus excavatum, the most frequent congenital chest wall deformity, may be rarely observed as a sole deformity or as a sign of an underlying connective tissue disorder. To date, only few studies have described correlations between this deformity and heritable connective tissue disorders such as Marfan, Ehlers-Danlos, Poland, MASS (Mitral valve prolapse, not progressive Aortic enlargement, Skeletal and Skin alterations) phenotype among others. When concurring with connective tissue disorder, cardiopulmonary and vascular involvement may be associated to the thoracic defect. Ruling out the concomitance of pectus excavatum and connective tissue disorders, therefore, may have a direct implication both on surgical outcome and long term prognosis. In this review we focused on biological bases of connective tissue disorders which may be relevant to the pathogenesis of pectus excavatum, portraying surgical and clinical implication of their concurrence.
Keywords: pectus excavatum; Marfan syndrome; MASS; fibrillin; fibrillinopathies pectus excavatum; Marfan syndrome; MASS; fibrillin; fibrillinopathies

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MDPI and ACS Style

Tocchioni, F.; Ghionzoli, M.; Messineo, A.; Romagnoli, P. Pectus Excavatum and Heritable Disorders of the Connective Tissue. Pediatr. Rep. 2013, 5, e15. https://doi.org/10.4081/pr.2013.e15

AMA Style

Tocchioni F, Ghionzoli M, Messineo A, Romagnoli P. Pectus Excavatum and Heritable Disorders of the Connective Tissue. Pediatric Reports. 2013; 5(3):e15. https://doi.org/10.4081/pr.2013.e15

Chicago/Turabian Style

Tocchioni, Francesca, Marco Ghionzoli, Antonio Messineo, and Paolo Romagnoli. 2013. "Pectus Excavatum and Heritable Disorders of the Connective Tissue" Pediatric Reports 5, no. 3: e15. https://doi.org/10.4081/pr.2013.e15

APA Style

Tocchioni, F., Ghionzoli, M., Messineo, A., & Romagnoli, P. (2013). Pectus Excavatum and Heritable Disorders of the Connective Tissue. Pediatric Reports, 5(3), e15. https://doi.org/10.4081/pr.2013.e15

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