A funnel-shaped chest (from Latin pectus excavatum), also known as "shoemaker", is the most common congenital defect of the chest wall. What is the cause of this deformation? What could be its he alth consequences? Do we have methods of its correction?
Funnel-shaped chest(shoemaker's chest, Latinpectus excavatum ) consists of a "funnel-shaped" hollow of the sternum, which is most often accompanied by deformation of adjacent ribs. Usually it is located at the bottom of the sternum. The concavity may be of various lengths and may be symmetrical or asymmetrical. The funnel-shaped chest constitutes as much as 90% of congenital anomalies of the chest wall. Its frequency is estimated at about 1 in 300-400 births. It occurs on average 3 times more often in boys.
Funnel-shaped chest: causes
The etiology of the shoemaker's chest is not fully understood. So far, there is no single documented causative factor, although the list of genes likely to be involved in its pathogenesis is gradually expanding. Its hereditary background may be proved by the fact that approx. 40% of patients suffering from this defect have at least one family member with the same defect. A funnel-shaped chest can also accompany diseases such as:
- Marfan's syndrome
- Poland's team
- Noonan band
- Ehlers-Danlos syndrome
It can also be a consequence of long-term rickets - a disease caused by vitamin deficiency. D.
Funnel chest: symptoms
A dimple in the anterior chest wall is usually noticed at birth (90% of cases are diagnosed within 1 year of life). The defect may get worse over time. It is related to the intensive growth of the skeleton in adolescence.
In the vast majority of cases, the funnel-shaped chest does not show significant clinical symptoms and is only a cosmetic problem, which, however, should not be underestimated. Lack of self-confidence, lack of body acceptance, feeling of shame and low self-esteem can cause psychological problems and even lead to depression in the future. This is true for both adolescent and adult patients.
Some patients may complain of discomfort or pain in the chest and back. Hashe is musculoskeletal in nature. It is worth mentioning that the shoemaker's cage may coexist with spine defects, e.g. scoliosis or excessive kyphosis.
The defect is sometimes so significant that the cavity causes pressure on the organs in the chest area - the lungs and the heart, the right ventricle of which lies in the immediate vicinity of its anterior wall. Severe forms can have serious clinical implications and cause, for example,
- reduced vital capacity of the lungs and, consequently, impaired exercise tolerance
- difficulty breathing
- recurrent respiratory infections
- dysfunction of the heart valves - mitral valve prolapse; less frequent regurgitation of the tricuspid and mitral valves
Fortunately, such advanced cases are found sporadically.
Funnel chest: diagnosis
The diagnosis of the funnel-shaped cage can be made with the naked eye, but in order to determine its severity and implement the necessary therapeutic measures, it is necessary to perform more detailed research. The basis is a radiological examination - chest X-ray and examination with computed tomography (CT). It is in the KT test that we can determine the so-called Haller's index - the ratio of the width of the chest to its depth (the shortest distance between the sternum and the spine). It allows for an objective assessment of the degree of severity of the defect - an index of more than 3.25 allows for a severe form that requires surgery (the standard is 2.5). It is also important to assess the functioning of the respiratory and circulatory systems - it is necessary to perform spirometry, ECG and ECHO of the heart. If a congenital syndrome accompanied by a shoemaker's cage is suspected, genetic testing may be necessary.
Funnel chest: treatment
Only surgery can provide complete correction of the defect! Currently, the Nuss method is the standard procedure in thoracic surgery. This procedure involves placing one or two metal plates of a convex shape under the bridge. After being pulled to the other side under camera control, they are then rotated 180 degrees along their long axis, which pushes the sternum forward. In this way, we obtain the desired effect. The plates are removed after the distortion has been corrected. This usually takes place after 2-3 years. The Nuss method is less invasive and is associated with a lower risk of recurrence than the previously used classic Ravitch procedure. It was associated with the removal of all deformed costal cartilages, cutting off the xiphoid process, and then stabilizationthe bridge in the correct position by means of a plate or a rod.
In the treatment of funnel chest, we can also distinguish non-surgical methods. In addition to corrective exercises, there are a number of more advanced activities. One of them is the use of the so-called vacuum bell - a pump that creates a vacuum to raise the sternum and reduce the degree of the defect. Another experimental method is to use a field generated by an array of two magnets - one implanted shallowly near the bottom of the sternum and the other worn on a special harness. The premise for the use of non-surgical methods is the compliance and flexibility of the chest skeleton, especially in the youngest patients. However, it should not be borne in mind that none of the methods of conservative treatment are still scientifically proven effective.
A relatively new procedure in the case of small, asymptomatic deformities are plastic surgery procedures. In order to fill the defect, which is the cavity on the anterior chest wall, the following are used, among others. specially modeled silicone implants or fatty tissue "transplanted" from other parts of the body. However, these are cosmetic procedures. They can be used, for example, in a residual defect after proper surgical correction. The basis of treatment is a repair procedure.