Abstract
Among all chest wall deformities Pectus Excavatum (PE) or funnel chest represents the most common congenital chest wall deformity accounting for 90 % of all deformities. The main characteristic is the depression of sternum and lower cartilages (Langer, Herrn JW Wiener med Zeit 49:515, 1880) with an incidence between 1 and 8 per 1000 children.
Access provided by Autonomous University of Puebla. Download chapter PDF
Similar content being viewed by others
Keywords
Pectus excavatum (PE) or funnel chest represents the most common congenital chest wall deformity accounting for 90 % of all deformities. Main characteristic is the depression of sternum and lower cartilages (Fig. 4.1, 4.2, and 4.3). The first description came from Bauhinus [1] in the sixteenth century. Another documented description of an appearance of the thorax could be found in 1860 by Woillez [2] and in 1863, von Luschka [3] reported about a 6-cm deep depression in the thorax wall of a 24-year-old man. Eggel [4] in 1870 published the first comprehensive case report of a patient with a funnel-formed thorax depression calling it a ‘miraculum naturae’. He assumed that the reason for the deformity would be a weakness and an abnormal flexibility of the sternum caused by nutritional disturbance or by developmental failure. Individual case reports followed by Williams [5], Flesch [6] and Hagmann [7]. The latter believed that overgrowth of the ribs causes the depression of the chest. Langer and Zuckerkandel [8] favoured the hypothesis of a developmental failure, taking place in utero, in which the lower jaw of the foetus is responsible for the deformity by pushing on the sternum as a result of too high intrauterine pressure. Meyer performed the first operation of PE in 1911 with the removal of the rib cartilage [9]. He also analysed the removed cartilage microscopically and identified an unspecific degeneration.
The incidence of PE has a ratio between 1 and 8 per 1000 children [10]. Interestingly, males are more often affected, with a gender distribution between 2:1 and 9:1 [11]. From the 19th century it has been recognised a genetic predisposition since a positive family history could be found in up to 43 % of PE cases [12, 13]. However, a specific genetic defect has not yet been found. Numerous syndromes are associated with PE and have been well described [14] where connective tissue disorder occurs in less than 1 % of all cases [15].
The majority of the patients with PE are tall, slim with associated scoliosis [14–16]. Severe depression of the sternum can cause displacement of the heart and reduction of lung volume [13, 17]. As a result of the anatomical changes, chest pain [12, 13, 15], fatigue [15], dyspnoea on exertion [12, 13, 15, 18], respiratory infections [13], asthma symptoms [13], palpitations [12] or heart murmurs could occur [13]. Several cases with mitral valve prolapse [13, 15, 19], mitral valve regurgitation and ventricle compression could be found [15, 17, 19]. For the latter, Coln [19] demonstrated that 95 % of 123 patients had cardiac compression. Even a single case report of syncopal symptoms has been reported. The pulmonary and cardiovascular functions of patients with PE deformities were analysed in many investigations and have revealed measurable deficiencies [12]. Fonkalsrud [13] reported that the symptoms of many untreated PE patients become progressively worse with age and he recommended an operational intervention for both young and adult patients.
In contrast to these descriptions of more or less severe clinical signs, symptoms affecting daily life activities are either rare [20]. Therefore, some authors described the indication for a PE correction to be primarily cosmetic.
Numerous clinical studies described an improvement of pulmonary and/or cardiovascular symptoms and improvement in the subjective well-being after surgical correction [9, 13, 16–18]. Malek [18, 21] concluded that an operative intervention improves cardiovascular but not pulmonary function. Guntheroth [22] and Spiers as well as Johnson [23] re-evaluated the source data of Malek’s meta-analyses and stated that due to relevant methodological deficits, these data failed to demonstrate any improvement of cardiac function. In this context, Aronson et al. [24] could not show an improvement in lung function parameters after Nuss procedure. Regardless the impact of the chest wall surgery to cardiopulmonary function the effect to psychological status of the patient is significant [15]. Numerous studies confirm that deformities cause relevant social discrimination, especially during adolescence, leading to the socio-psychologic problems [15]. A multicentre study demonstrated that the surgical repair of PE patients improves these socio-psychologic problems. [25–32]
References
Bauhinus J. Johannes Observatorium medicarum, rararum, novarum, admirabilium, et montrosarum, liber secundus. In: Ioannis Schenckii a Grafenberg, editor. Observation. Frankfurt: De partibus vitalibus, thorace contentis; 1609. p. 322.
Woillez. Sur un cas de deformitée thoracique considérable avec deplacement inoffensif de plusieur organes et signes sthetoscopiaques particulières. Paris: Rap Soc Med d’Hop; 1860. p. 3.
von Luschka H. Die Anatomie der Brust des Menschen. Die Anatomie des Menschen in Rücksicht auf die Bedürfnisse der praktischen Heilkunde. Tübingen: Laupp; 1863. p. 23
Eggel. Eine seltene Mißbildung des Thorax. Virchows Arch Path Anat. 1870;49:230.
Williams CT. Congenital malformation of the thorax great depression of the sternum. Trans Path Soc. 1872;24:50.
Flesch M. Über eine seltene Missbildung des Thorax. Virchows Arch Path Anat. 1873;75:289.
Hagmann. Selten vorkommende Abnormität des Brustkastens. Jb Kinderheilkunde. 1888;15:455.
Langer E. Zuckerkandel: Untersuchungen über den mißbildeten Brustkorb des. Herrn JW Wiener med Zeit. 1880;49:515.
Meyer L. Zur chirurgischen Behandlung der angeborenen Trichterbrust. Berl Klin Wschr. 1911;48:1563–6.
Kelly Jr RE, Lawson ML, Paidas CN, Hruban RH. Pectus excavatum in a 112-year autopsy series: anatomic findings and the effect on survival. J Pediatr Surg. 2005;40:1275–8.
Ravitch MM. Repair of pectus excavatum in children under 3 years of age: a twelve-year experience. Ann Thorac Surg. 1977;23:301.
Kelly Jr RE, Shamberger RC, Mellins RB, Mitchell KK, Lawson ML, Oldham K, et al. Prospective multicenter study of surgical correction of pectus excavatum: design, perioperative complications, pain, and baseline pulmonary function facilitated by internet-based data collection. J Am Coll Surg. 2007;205:205–16.
Fonkalsrud EW, Dunn JC, Atkinson JB. Repair of pectus excavatum deformities: 30 years of experience with 375 patients. Ann Surg. 2000;231:443–8.
Kotzot D, Schwabegger AH. Etiology of chest wall deformities—a genetic review for the treating physician. J Pediatr Surg. 2009;44:2004–11.
Colombani PM. Preoperative assessment of chest wall deformities. Semin Thorac Cardiovasc Surg. 2009;21:58–63.
Beiser GD, Epstein SE, Stampfer M, Goldstein RE, Noland SP, Levitsky S. Impairment of cardiac function in patients with pectus excavatum, with improvement after operative correction. N Engl J Med. 1972;287:267–72.
Kubiak R, Habelt S, Hammer J, Hacker FM, Mayr J, Bielek J. Pulmonary function following completion of Minimally Invasive Repair for Pectus Excavatum (MIRPE). Eur J Pediatr Surg. 2007;17:255–60.
Malek MH, Berger DE, Housh TJ, Marelich WD, Coburn JW, Beck TW. Cardiovascular function following surgical repair of pectus excavatum: a metaanalysis. Chest. 2006;130:506–16.
Coln E, Carrasco J, Coln D. Demonstrating relief of cardiac compression with the Nuss minimally invasive repair for pectus excavatum. J Pediatr Surg. 2006;41:683–6.
Luzzi L, Voltolini L, Zacharias J, Campione A, Ghiribelli C, Di Bisceglie M, et al. Ten year experience of bioabsorbable mesh support in pectus excavatum repair. Br J Plast Surg. 2004;57:733–40.
Malek MH, Berger DE, Marelich WD, Coburn JW, Beck TW, Housh TJ. Pulmonary function following surgical repair of pectus excavatum: a meta-analysis. Eur J Cardiothorac Surg. 2006;30:637–43.
Guntheroth WG, Spiers PS. Cardiac function before and after surgery for pectus excavatum. Am J Cardiol. 2007;99:1762–4.
Johnson JN, Hartman TK, Pianosi PT, Driscoll DJ. Cardiorespiratory function after operation for pectus excavatum. J Pediatr. 2008;153:359–64.
Aronson DC, Bosgraaf RP, Merz EM, van Steenwijk RP, van Aalderen WM, van Baren R. Lung function after the minimal invasive pectus excavatum repair (Nuss procedure). World J Surg. 2007;31:1518–22.
Kelly Jr RE, Cash TF, Shamberger RC, Mitchell KK, Mellins RB, Lawson ML, et al. Surgical repair of pectus excavatum markedly improves body image and perceived ability for physical activity: multicenter study. Pediatrics. 2008;122:1218–22.
Brown AL. Pectus excavatum (funnel chest). J Thorac Surg. 1939;9:164–84.
Sweet RH. Pectus excavatum: report of two cases successfully operated upon. Ann Surg. 1944;119:922–34.
Fokin AA, Robicsek F, Watts LT. Genetic analysis of connective tissue in patients with congenital thoracic abnormalities. Interact CardioVasc Thorac Surg. 2008;7:56.
Nakaoka T, Uemura S, Yano T, Nakagawa Y, Tanimoto T, Suehiro S. Does overgrowth of costal cartilage cause pectus excavatum? A study on the lengths of ribs and costal cartilages in asymmetric patients. J Pediatr Surg. 2009;44:1333–6.
Geisbe H, Buddecke E, Flach A, Muller G, Stein U. 88. Biochemical, morphological and physical as well as animal experimental studies on the pathogenesis of funnel chest. Langenbecks Arch Chir. 1967;319:536–41.
Rupprecht H, Hummer HP, Stoss H, Waldherr T. Pathogenesis of chest wall abnormalities—electron microscopy studies and trace element analysis of rib cartilage. Z Kinderchir. 1987;42:228–9.
Feng J, Hu T, Liu W, Zhang S, Tang Y, Chen R, et al. The biomechanical, morphologic, and histochemical properties of the costal cartilages in children with pectus excavatum. J Pediatr Surg. 2001;36:1770–6.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Kolvekar, S.K., Panagiotopoulos, N. (2016). Pectus Excavatum. In: Kolvekar, S., Pilegaard, H. (eds) Chest Wall Deformities and Corrective Procedures. Springer, Cham. https://doi.org/10.1007/978-3-319-23968-2_4
Download citation
DOI: https://doi.org/10.1007/978-3-319-23968-2_4
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-23966-8
Online ISBN: 978-3-319-23968-2
eBook Packages: MedicineMedicine (R0)