Generality
The pectus excavatum it is a defect of the rib cage, characterized by a depression in the center of the thorax.
Figure: Severe case of fart excavatum in a young patient
The reasons that lead to the onset of this almost always congenital malformation, also known as pectus excavatum or funnel chest, are unclear; according to some researchers, it could be a hereditary anomaly, transmitted from parents to children.
The chest hollow gets worse with growth.
Except for the aesthetic discomfort, the pectus excavatum it is usually asymptomatic; only the most severe forms cause significant compression of the heart and lungs, which leads to cardio-circulatory and respiratory problems.
Therapy is generally non-surgical; surgery, in fact, is reserved only for the most serious cases.
What is pectus excavatum?
The pectus excavatum is an "anomaly of the rib cage characterized by a hollowing of the central part of the sternum. In fact, people who are affected they have a particularly evident hollow in the center of the thorax.
Almost always congenital, the anomaly is also known as excavated or funnel chest (or thorax).
What does the term congenital mean?
A disease is defined as congenital when it is present from birth. A congenital defect can be transmitted by inheritance (i.e. from parents to child) or in a completely sporadic way.
PECTUS EXCAVATUM IS IT A SERIOUS DEFECT?
Beyond the aesthetic discomfort it creates in many affected subjects, the pectus excavatum (especially when very accentuated) can cause cardio-circulatory and respiratory problems.
EPIDEMIOLOGY
The pectus excavatum it is the most common deformity of the anterior part of the thorax: in fact, about 90% of the sternal deformities (ie of the sternum) are represented by the pectus excavatum.
From a statistical study, it was found that 40% of subjects with a funnel chest have at least one immediate family member with the same thoracic anomaly.
Male people are certainly more affected than female people (the ratio is 3 to 1); in this regard, some accredited sources report that the frequency of the funnel chest, among male children, is equal to one case for every 300-400 new borns.
Causes
The precise causes of the pectus excavatum are, at the moment, unclear.
For those with a family history of pectus excavatum, it is conceivable that one or more inherited genetic mutations are at the origin of the anomaly.
However, this hypothesis still has some open points, such as the identification of the gene or genes responsible and the mode of transmission.
WHAT IS THE PROCESS THAT LEADS TO INFOSSATURA THE STERNO?
As the causes of origin, also the process of sinking of the sternum is little known.
Scientists suspect that it is due to an excessive growth of the cartilage that connects the ribs to the breastbone. It seems, in fact, that this abnormal cartilage growth pushes inwards (towards the spine) the central area of the chest.
PECTUS EXCAVATUM AND ASSOCIATED DISEASES
The pectus excavatum it represents a possible pathological sign of some morbid states. Such diseases are:
- Marfan's syndrome. It is a genetic disease affecting the connective tissue, which causes musculoskeletal deformations, cardiovascular problems, visual impairment, dyspnoea and other pulmonary disorders, facial changes, language difficulties and finally particular skin signs. Being a genetic disease, Marfan's syndrome is incurable; therefore there are no drugs or other types of therapies that can solve the disease. The only treatments available relieve the symptoms and improve the health of the patient.
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Figure: pathological signs characteristic of rickets. The term craniotabe refers to a weakening (or softening) of the occipital bone and the two parietal bones of the skull. Harrison's sulcus is a horizontal depression of the last ribs. The rachitic rosary is a swelling of the chondrocostal joints (made up of cartilage tissue ). Rickets. Rickets is a skeletal disease (osteopathy) that begins in childhood and depends on a defect in the mineralization of the bone matrix.The cause of this defect is the lack of vitamin D, very often associated with low calcium intakes with the diet and / or reduced exposure to sunlight. In an advanced stage, rickets causes bone deformities and fractures.
- Scoliosis. It is an "abnormal curvature of the spine.
However, in most cases, the pectus excavatum it is an "anomaly in its own right, not accompanied by any other disorder or pathology.
Symptoms and Complications
The hallmark of the pectus excavatum is the sunken sternum. The depth of the hollow usually tends to worsen over the course of life: it appears shortly after birth and undergoes a "accentuation" during adolescence and adulthood.
However, except for this sign, the pectus excavatum it is generally asymptomatic (i.e. without obvious symptoms).
WHEN THE PECTUS EXCAVATUM IS IT CAUSE OF SYMPTOMS? COMPLICATIONS
When the pectus excavatum it is particularly severe, it is possible that the sunken sternum compresses the heart and lungs. Compression of the heart and lungs impairs the normal functioning of these organs and could lead to the appearance of:
- Reduced tolerance to physical exercise and efforts in general
- Faster heart rate (tachycardia or palpitation)
- Recurrent respiratory infections
- Chest pain
- Murmurs or other heart "noises"
- Sense of fatigue
- Wheezing or coughing
PECTUS EXCAVATUM AND AESTHETIC DISCOMFORT
Many individuals with a funnel chest they also have a curved back and protruding ribs and shoulder blades.
These anomalies often involve a certain aesthetic discomfort. In fact, people who suffer from it are ashamed of their physical appearance and tend to avoid all motor activities in which they have to take off their clothes (such as swimming).
Diagnosis
To diagnose the pectus excavatum, a physical examination is usually sufficient, during which the doctor analyzes the appearance of the chest and measures the hollow of the breastbone.
To determine, however, whether the pectus excavatum it is severe to the point of affecting the proper functioning of the heart and lungs, the doctor must resort to other diagnostic tests, including:
- Chest X-ray (Chest X-ray)
- CT scan (or computed axial tomography)
- Electrocardiogram (or ECG)
- Echocardiogram
- Respiratory function tests
What is the referring doctor in case of severe pectus excavatum?
Typically, to deal with more severe cases of pectus excavatum, are doctors who are experts in thoracic surgery.
CHEST X-RAY
Chest x-ray is a diagnostic X-ray imaging examination, which allows the visualization of the main anatomical structures of the thorax: therefore the heart, lungs, most important blood vessels, most of the ribs, the sternal bone and a portion of the spine vertebral.
It is a painless procedure, but still considered minimally invasive, as it exposes the patient to a certain dose of harmful ionizing radiation.
CT scan
The CT scan is another diagnostic imaging test, which allows a fairly clear view of the internal organs (N.B: in this case, the organs viewed are the organs present in the thoracic level).
During its execution, the patient is exposed to a minimal amount of harmful ionizing radiation. Therefore, the test is considered invasive.
The Haller index, measured by CT scan, is an index that relates the width of the rib cage and the distance between the sternum and the spine. A high value of the Haller index means severe pectus excavatum.
A Haller index of 2.5 is typical of a normal chest; on the contrary, a Haller index of 3.2 is characteristic of pectus excavatum serious.
ELECTROCARDIOGRAM
The ECG measures the electrical activity of the heart through the application, on the chest and limbs, of some electrodes. Based on the recording of how the signal for cardiac contraction travels, the cardiologist is able to identify any anomalies of the rhythm cardiac (tachycardia, arrhythmias, etc.).
The ECG is a simple examination, does not require any special preparation and is not invasive.
ECHOCARDIOGRAM
The "echocardiogram is an ultrasound examination that shows, in detail, the anatomy of the heart and any anomalies of the latter." It allows, in fact, to highlight the defects of the septum that separates the atria and ventricles, the valve defects, the malformations of the myocardium (i.e. the heart muscle), the pumping difficulties and compressions that the heart undergoes due to the effect of the adjacent anatomical structures.
The echocardiogram is a simple and non-invasive examination, which involves the use of an ultrasound probe (transducer) placed on the patient's chest.
TESTS OF RESPIRATORY FUNCTIONALITY
The most performed respiratory function tests are:
- Spirometry. Fast, practical, and painless, spirometry records the inspiratory and expiratory capacity of the lungs and evaluates the opening of the airways passing through them.
- The stress test. It consists in evaluating how the heart rate, blood pressure and breathing of the patient engaged in a more or less intense physical activity vary.
Treatment
The anatomical defect due to the pectus excavatum it can be resolved by surgery.
However, it must be remembered that surgery usually occurs only when the hollow in the sternum is very severe. Reasoning in terms of Haller index, the potential candidates for surgery are subjects with a value equal to or greater than 3, 25.
In case of slight sternal hollows, surgery is only considered for aesthetic reasons; in these cases, in fact, doctors prefer not to intervene in any way or do it through simple physiotherapy.
Recently, a particular therapeutic tool has been developed, called vacuum bell.
PHYSIOTHERAPY
Physiotherapeutic treatments consist of stretching and muscle strengthening exercises, which should correct posture and slow down the sinking process.
Physiotherapy works best in young people, as their bone growth is still moldable.
VACUUM BELL
Figure: a vacuum bell. From wikipedia.org
There vacuum bell it is a bell-shaped suction cup, connected to a pump and constructed in such a way as to be applied in correspondence with the sternal cavity.
Positioned on the chest in the appropriate point, due to the "vacuum" effect generated inside the suction cup (thanks to the pump), the vacuum bell would seem to reduce the severity of the pectus excavatum.
According to those who believe that the treatment with vacuum bell is effective, to obtain appreciable results the therapy must in any case last many months or years.
As for the contraindications: in the short term there do not seem to be any; while in the long term they are still to be determined.
There vacuum bell it is an invention of a German engineer named Eckart Klobe.
Attention: doctors advise against the use of vacuum bell in case of angiopathies, aneurysm of the aorta, haemophilia and osteoporosis.
SURGERY
The surgery for the correction of the pectus excavatum it can be done in various ways. The two main methods are:
- Ravitch's technique. It is a "traditional surgery operation during which the surgeon makes an" incision of several centimeters on the chest and intervenes "in the open".
The method basically consists in removing part of the sternal cartilage and realigning the sternum, with the help of a special metal tool. The metal tool, which acts as a real corrective tool, is left in place for at least 6-12 months: this is the time it takes for the cartilage to reform and for the sternum to calcify in the correct position. - The Nuss procedure. It is laparoscopic surgery. Its execution involves the making of two small incisions on the sides of the chest. Through these two incisions, the surgeon inserts an instrument equipped with a camera to orient himself inside the thorax and apply one or more metal bars to it. for the correction of sternal deformity.
The procedure has the advantage of being minimally invasive, but has the disadvantage that the metal bars must be held in place for approximately 24 months.
The two techniques compared
The Nuss procedure is less dangerous than the traditional intervention; moreover, it has the following advantages:
- It is less painful (especially during the post-operative phase)
- It provides for a "shorter hospitalization
- It has faster recovery times
- It leaves less noticeable scars
Nevertheless, the Ravitch technique still represents a good solution, as the corrective metal instrument can be removed already after 6 months from the intervention.
Prognosis
Except in its most severe forms, the pectus excavatum it is a minor anatomical defect, which does not affect the life of those affected.