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Pectus Carinatum

Pectus Carinatum (PC) is a thoracic deformity in which an abnormal costal cartilage growth produces an outward anterior chest wall protrusion described as “pigeon chest”.

PC  is considered the second-most common cause of thoracic malformation after pectus excavatum (PE), with a reported incidence ranging from a fifth of PE in some centres, to arrive to an equal incidence in others. It is more prevalent in males with a ratio of 3-4 to 1.

PC is often noted in childhood with progression during adolescence.

 

PC malformations could be classified according to the shape of the protuberance in symmetric (photo 1) and asymmetric defect (photo 2) and in chondrogladiolar or chondromanubrial defect depending if the site of sternal angulation is located at the body of the sternum or at the manubrium. The chondrogladiolar is the more common one.

PC can be also a secondary malformation, occurring after sternotomies for cardiac surgery or following surgical PE repair.

 

Diagnosis is made by physical examination and the elasticity of the sternum and related deformed costal cartilages can always be assessed by manual compression or by means of pressure-measuring devices.

PC1.jpg

   Photo 1: Symmetric PC                                                           Photo 2: Asymmetric Pcù

 

Author

Antonio Messineo, M.D. 

References

Mantinez-Ferro et al. Seminar in Ped. Surgery. 2008

De Beer et al. Ann. Thorac. Surgery. 2017 

Fraser et al. Ann. Thorac. Surgery. 2020

Quality of Life

 

Pectus carinatum often results in a reduced quality of life (QoL) due to a disturbed body image and lower self-esteem. Several studies have shown a significant reduction of QoL. After correction of the deformity, pectus patients had a significant improvement of QoL.

Author

Caroline Fortmann, M.D.

 

References

Paulson et al. J Pediatr Surg. 2019

Bostanci et al. J Pediatr Surg. 2013

Knudsen et al. J Pediatr Surg. 2015

 

Bracing

 

Over last years, bracing for pectus carinatum has gained significant popularity, and this has been corroborated by the increasing number of publications on bracing versus surgery. However, this form of therapy was not new as the first reports were published almost three decades ago. Most recent reviews, endorse bracing as first line treatment for pectus carinatum and it has demonstrated to be effective to treat both, symmetric and asymmetric cases.

A wide range of commercially available braces are available, some of them include pressure measuring capabilities although local orthotics departments are also capable of creating their own pectus carinatum braces.

It is highly likely that a bracing program is more important than the brace itself and prescribing a brace alone is unlikely to result in success. Continued follow-up during treatment, with frequent adjustments, assessment of patient progress, and maintenance of patient motivation, is essential.

Fig 1. Symmetric Pectus Carinatum: Results after 9 months of treatment of a 13-year-old patient with severe symmetric pectus carinatum using a Dynamic Compression System. Pressure of initial correction (PIC) 6,5 PSI
Fig 2. Asymmetric Pectus Carinatum: Results after one year of treatment with late follow-up pictures (2.5 years) of a 14-year-old patient with severe asymmetric pectus carinatum using a Dynamic Compression System. Pressure of initial correction (PIC) 5,5 PSI
 

Author

Marcelo Martinez-Ferro, M.D.

References

Haje et al. J Pediatr Orthop 1992

Martinez-Ferro et al. Semin Pediatr Surg. 2008 

Emil et al. Eur J Pediatr Surg. 2018 

de Beer et al. Semin Pediatr Surg. 2018

 

Modified Ravitch Procedure

In the case of chondrogladiolar PC, the general concept of the modified Ravitch operation also applies. Once a subperidondrial resection of all deformed cartilage has been obtained, and the sternum has been corrected at the same level as the insertions of the intercostal muscles, one or two osteotomies of the anterior table can be performed if necessary, allowing the sternum to be fractured and displaced posteriorly, almost exactly as we explained for PE.

In cases of a chondromanubrial deformity (see also “Currarino-Silverman-Syndrome” in the subgroup “others”), a titanium plate is also recommended to repair the transverse osteotomy of the sternum, with which it is possible to obtain adequate correction and a better position of the chest wall (Figure 1a & b). In most cases the resection can be a larger triangle than a simple transverse osteotomy

Figure 1a & b: System of parallel plates to repair the transverse osteotomy of the sternum. This allows the young patient to have a sternotomy, if it is ever necessary.

 

To finish the procedure, the pectoralis major muscles have to be brought together, which requires a submuscular suction drainage for a few hours, and closure of the subcutaneous tissue and skin with running sutures.

 

Author

José Ribas Milanez de Campos, M.D.

 

References

Ravitch. J Thorac Surg. 1952

Sulamaa et al. Acta Chir Scand. 1959

Haller et al. Ann Surg. 1976

Mao et al. J Pediatr Surg. 2017

Shamberger et al. J Pediatr Surg. 1988

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