Generality
Pectus excavatum is a defect of the thoracic cage, characterized by a hollow in the center of the chest.
Figure: Severe case of a fart excavated in a young patient
The reasons that lead to the onset of this malformation almost always congenital, also known as an excavated chest or a funnel-shaped chest, are unclear; according to some researchers, it could be a hereditary anomaly, transmitted from parents to children.
The thoracic depression is getting worse with growth.
Except for aesthetic discomfort, pectus excavatum is usually asymptomatic; only the most severe forms cause an important compression of the heart and lungs, which leads to the appearance of 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?
Pectus excavatum is an anomaly of the rib cage characterized by a hollowing of the central part of the sternum. In fact, the people who are affected they have a noticeable hollow in the center of the chest.
Almost always congenital, the anomaly is also known as the excavated or funneled chest (or chest) .
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 (ie from parents to child) or in a completely sporadic way.
IS PECTUS EXCAVATUM A SERIOUS DEFECT?
Beyond the aesthetic discomfort that creates in many affected subjects, the pectus excavatum (especially when very pronounced) can cause cardio-circulatory and respiratory problems.
Epidemiology
Pectus excavatum is the most common deformity of the front of the chest: in fact, about 90% of the sternal deformations (ie of the sternum) is represented by the hollowed chest.
From a statistical study, it was found that 40% of subjects with a funnel-shaped chest had at least one 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 breast funnel, among male children, is equal to one case every 300-400 newborns.
Causes
The precise causes of the pectus excavatum are, at the moment, unclear.
For those who have a family history of excavated chest, it is conceivable that at the origin of the anomaly there are one or more genetic mutations transmitted in a hereditary way.
However, this hypothesis still has some pending points, such as the identification of the gene or genes responsible and the modes of transmission.
WHAT IS THE PROCESS THAT LEADS TO THE SIDE OF THE STERN?
Like the causes of origin, the process of sinking the sternum is also little known.
Scientists suspect that it is due to excessive growth of the cartilage that connects the ribs to the sternum. Indeed, it would seem that this abnormal cartilage growth pushes the central area of the thorax inward (towards the spine).
PECTUS EXCAVATUM AND ASSOCIATED DISEASES
Pectus excavatum represents a possible pathological sign of some morbid states. These diseases are:
- Marfan's syndrome . It is a genetic disease affecting the connective tissue, which causes musculoskeletal deformations, cardiocirculatory problems, visual deficits, dyspnea and other pulmonary disorders, facial alterations, language difficulties and, finally, particular cutaneous signs. Being a genetic disease, Marfan syndrome is incurable; therefore there are no drugs or other types of therapies that can resolve the disease. The only treatments available alleviate the symptoms and improve the patient's health.
- Scoliosis . It is an abnormal curvature of the spine.
However, in most cases, pectus excavatum 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 depression usually tends to worsen over a lifetime: it appears shortly after birth and undergoes an accentuation during adolescence and adulthood.
However, except for this sign, the pectus excavatum is generally asymptomatic (ie free of obvious symptoms).
WHEN DOES THE PECTUS EXCAVATUM CAUSE SYMPTOMS? COMPLICATIONS
When the pectus excavatum is particularly severe, it is possible that the sunken sternum will compress the heart and lungs. The compression of the heart and lungs affects the normal functioning of these organs and could lead to the appearance of:
- Reduced tolerance to exercise and efforts in general
- Acceleration of heart rate (tachycardia or palpitation)
- Recurrent respiratory infections
- Chest pain
- Puffs or other cardiac "noises"
- Sense of fatigue
- Wheezing or coughing
PECTUS EXCAVATUM AND AESTHETIC DISCOMFORT
Many individuals with funnel chest they also have a curved back and protruding ribs and shoulder blades .
Such anomalies often involve a certain aesthetic discomfort. In fact, the people who are affected are ashamed of their physical appearance and tend to avoid all the motor activities in which they have to take off their clothes (such as swimming).
Diagnosis
To diagnose the excavated chest, the physical examination is usually sufficient, during which the doctor analyzes the appearance of the chest and measures the sternum hollow.
To determine, however, if the pectus excavatum 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 computerized axial tomography)
- Electrocardiogram (or ECG)
- Echocardiogram
- Respiratory function tests
What is the doctor of reference in case of severe pectus excavatum ?
In general, physicians experienced in thoracic surgery deal with the most severe cases of pectus excavatum .
CHEST X-RAY
The RX-thorax is a radiological imaging diagnostic examination, which allows the visualization of the main anatomical structures of the thorax: therefore heart, lungs, most important blood vessels, most of the ribs, the sternal bone and a portion of the column vertebral.
It is a painless procedure, but still considered minimally invasive, since it exposes the patient to a certain dose of harmful ionizing radiation .
TAC
The CT scan is another diagnostic imaging test, which allows a fairly clear visualization of the internal organs (NB: in this case, the organs viewed are the organs present at the thoracic level).
During its execution, the patient is exposed to a minimum amount of harmful ionizing radiation. Therefore, the test is to be considered invasive.
The Haller index, measured by TAC, is an index that relates the width of the rib cage and the distance between the sternum and the vertebral column. 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 grave.
ELECTROCARDIOGRAM
The ECG measures the electrical activity of the heart by applying some electrodes to the chest and limbs. Based on the recording of how the heart contraction signal travels, the cardiologist is able to identify any abnormal heart rhythm (tachycardia, arrhythmias, etc.).
The ECG is a simple exam, requires no special preparation and is not invasive.
ECHOCARDIOGRAM
The echocardiogram is an ultrasound examination that shows, in detail, the anatomy of the heart and the possible anomalies of the latter. It allows, in fact, to highlight the defects of the septum that separates atriums and ventricles, valve defects, myocardial malformations (ie the heart muscle), pumping difficulties and compressions that the heart undergoes due to the adjacent anatomical structures.
The echocardiogram is a simple and non-invasive examination, which involves the use of an ultrasound probe (transducer) resting on the patient's chest.
FUNCTIONALITY TESTS? RESPIRATORY
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 assessing how the patient's heart rate, blood pressure and breathing vary in a more or less intense physical activity.
Treatment
The anatomical defect due to pectus excavatum can be resolved by surgery.
However, it should be remembered that surgery is usually used only when the hollow in the sternum is very severe. Reasoning in terms of the Haller index, the potential candidates for the surgical operation are the subjects with a value equal to or greater than 3.25.
In the case of slight sternal bumps, surgery is only considered for aesthetic reasons ; in these cases, in fact, doctors prefer not to intervene in any way or do it by simple physiotherapy.
Recently, a special therapeutic tool has been developed, called vacuum bell .
PHYSIOTHERAPY
Physiotherapy treatments consist of stretching and muscle strengthening exercises, which should correct the posture and slow down the process of the sternum sinking.
Physiotherapy has better effects in young subjects, as their bone growth is still mouldable.
VACUUM BELL
Figure: a vacuum bell. From wikipedia.org
The vacuum bell is a bell -shaped suction cup, connected to a pump and constructed in such a way as to be applied at the sternum recess.
Positioned on the chest in the appropriate point, due to the "empty" 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, the therapy must last for many months or years to obtain appreciable results.
Regarding the contraindications: in the short term there do not seem to be any; while in the long term they are still to be established.
The vacuum bell is an invention of a German engineer named Eckart Klobe .
Warning: doctors advise against the use of the vacuum bell in case of angiopathies, an aortic aneurysm, hemophilia and osteoporosis.
SURGERY
Surgery for correcting pectus excavatum can be performed in various ways. The two main methods are:
- The Ravitch 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 consists substantially in removing part of the sternal cartilage and in realigning the sternum, with the help of a special metallic instrument. The metallic instrument, 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 itself and the sternum to calcify in the correct position.
- The Nuss procedure . It is laparoscopic surgery. Its execution involves the creation of two small incisions on the sides of the chest. Through these two incisions, the surgeon inserts an instrument equipped with a video camera to orientate himself inside the chest and apply one or more metal bars for the correction of the sternal deformity.
The procedure has the advantage of being minimally invasive, but has the drawback that the metal bars must be held in position for about 24 months.
The two techniques compared
The Nuss procedure is less dangerous than the traditional intervention; furthermore, it has the following advantages:
- It is less painful (especially during the post-operative phase)
- Provides shorter hospitalization
- It has faster recovery times
- Leaves less noticeable scars
Nevertheless, the Ravitch technique is still a good solution, as the corrective metal tool can be removed as early as 6 months after surgery.
Prognosis
Except in its most severe forms, the pectus excavatum is a mild anatomical defect, which does not affect the life of those affected.
