What Is Pectus Excavatum?

Pektus Ekskavatum Nedir?

Pectus Excavatum is the most frequently seen chest wall deformity. Chest wall deformities are classified in five categories consisting of Pectus Excavatum, Pectus Carinatum, Poland Syndrome, Sternal defects, and the uncommon chest wall deformities (1). 85% of the deformities can be detected in the first year of life.

Pectus Excavatum can be called as “sunken or hollowed chest” (Figure 1).

Pectus Excavatum can be seen together with Pectus Excavatum pectus carinatum (PC) in 6.8% of the patients which is called “mixed deformity”.

 

Pectus Excavatum is the most frequent deformity among these five categories. It is seen in 1/300-1/400 live births. It is not uncommon as assumed. It is seen 4-fold more in males than females. There is an increased family predisposition in Pectus Excavatum patients – a rate of 37% family history exist.

Despite the cause of the deformity is due to the excessive growth of the cartilage ribs, absolute cause is not being known exactly. Irregular grown part of the cartilage pulls the sternum back. This is not a big problem in the childhood Pectus Excavatum period. But, as the patient grows, it causes pain at the disorganized cartilage ribs and around the left breast. After the physical exercise with effort, it might cause palpitation, rhythm problem, and murmur at the heart.

In general, the Pectus Excavatum does not have any harmful impact on the interior organs. The excessive back displacing of the chest wall might cause collapse on the right ventricle of the heart. This extreme displacement of the chest wall mostly pushes the heart to the left. This collapse on the heart improves after the correction of the sternal deformity. In brief, volume of the space being stolen from the heart and the lungs is equal to the volume of the collapse created by the chest wall deformity on these organs. The severity of the deformity should be evaluated correctly for the sake of successful surgical correction.

Figure 1 –"Anterior appearance" of a patient with sunken chest (Pectus Excavatum)"

Some other diseases may accompany the Pectus Excavatum deformity;

The severity of the deformity is worse in patients with Marfan Syndrome. The male patients with Pectus Excavatum and scoliosis (lateral tortuosity of the backbone) should also be assessed for the Marfan Syndrome. Echocardiography should be obtained; extension in the aortic root and back flow of the blood ('murmur' can be heard) through the aortic and mitral valves (the valve staying between left auricle and the left ventricle) would support the diagnosis of the Marfan Syndrome.

TOF (Tetralogy of Fallot – a congenital heart disease) and MVP (Mitral Valve Prolapse) can be seen with Pectus Excavatum (2).

Pectus Excavatum can be seen frequently in patients with muscle-skeleton diseases and developmental abnormalities. Pectus Excavatum can also be seen with the connective tissue diseases (Ehlers Danlos, Marfan Syndrome, Ostegenesis Imperfecta with Pectus Excavatum, etc ) and homosistinuria (3). It may be detected with Down syndrome, TOF, and congenital diaphragmatic hernia. In 5% to 25% of the Pectus Excavatum patients, scoliosis (lateral tortuosity of the backbone) and kyphoscoliosis (humpbacked) accompany to the deformity (4).

Brief History for the Pectus Excavatum treament;

First surgical correction for the Pectus Excavatum had been performed in 1911 and 1913 by Meyer and Sauerbruch. Ravitch had defined the technique which has been modified since then in 1949. This technique includes;

1-) total excision (cutting) of the whole deformed cartilage ribs with their own pericondrium (sheath),

2-) total excision of the xyphoid (the lower tip of the sternum) from the body of the sternum,

3-) separation of the intercostal bands from the sternum,

4-) transverse excision of the sternum.

This technique allows to displace the sternum forward by supporting the back part of the sternum with a Kirchner wire. By this technique, the sternum comes to a normal position which is supposed to be.
Baronofsky and Welch have described a technique in 1957 and 1958, respectively. The main procedures include keeping the sheath of the deformed cartilage ribs and the intercostal bands between the upper ribs intact, excision of the sternum, and fixing the sternum forward by the silk sutures.

Besides, Haller had portrayed 'Tripod Fixing System' in 1957. This system includes excision of the back surface of the sternum, removing the deformed cartilage ribs under their sheaths, and oblique division of the normal 2nd and 3rd ribs from back to forward. This method had succeeded at a rate of 100% in 45 patients with Pectus Excavatum deformity.

And also, 'sternal reversal technique' had been described (sternal turnover). It had been performed in Japan for the first time. The sternum had been turned reverse freely for 180° and then fixed to the cartilage ribs by heavy sutures. This radical technique has not been used widely because of high complication rates.

The recent widely accepted and used technique has been described by Prof. Donald Nuss in 1987 for the first time (5). This minimal invasive technique is a surgical technique being commonly used nowadays with minimal invasive surgical technique, short procedure time, fast recovery span after the procedure, and excellent long term results.
It is being used very commonly since it was first described in 1987.


References

(1) Shields TW: General Thoracic Surgery, Baltimore/ Philadelphia, Williams&Wilkins, 1994, p. 529- 557
(2) TGKDC, July 1998, 6/4, s. 357–361
(3) Chest. 1974;66.165–170
(4) Paedeatric Respiratory Reviews. 2003;4:237–242; J Pediatr Surg. 2005;40:174–180
(5) J Pediatr Surg. 1998;33:545–552

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Erkan YILDIRIM, MD, FETCS

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