About Pectus Excavatum
AT THE FOREFRONT OF PECTUS EXCAVATUM TREATMENT
What is Pectus Excavatum?
What is Pectus Excavatum? Pectus Excavatum is the most frequent congenital deformity of the thoracic cavity, accounting for almost 95% of cases. It is characterized by a concave depression of the sternum that gives a sunken appearance to the chest, also known as funnel chest. The malformation is diagnosed in newborns and develops progressively during puberty. It is estimated that it occurs approximately in one of every 500-1000 births and, in general, it is more frequent among males (male:female ratio=3:1). The opposite deformity, also common among thoracic deformities, is known as Pectus Carinatum.
The etiology of Pectus Excavatum remains unknown, although it can be considered a hereditary condition. There is a strong suspicion that it is an intrinsic disease at the molecular level of the cartilage of the costal sternal joints in variable number and intensity. On rare occasions, PE is syndromic (Marfan, Morquio, Pierre Robin, Prune Belly, Poland, Jeune, etc.), although it usually occurs in isolation.
AN ASSESSMENT BY A PEDIATRIC OR THORACIC SURGEON IS IMPORTANT TO RULE OUT ANY CHEST WALL PATHOLOGY. CONSULT YOUR PEDIATRICIAN OR FAMILY DOCTOR.
DO YOU HAVE PECTUS EXCAVATUM?
DO YOU HAVE PECTUS EXCAVATUM?
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Diagnosis of Pectus Excavatum
Being a pathology that usually exacerbates with age, early assessment by an expert surgeon is very important.
This deformity is progressive and usually makes its appearance in early childhood, although it can be visible from birth. The pediatrician or family doctor is usually the first professional consulted by patients to request information about Pectus Excavatum.
Many of the people affected by this pathology lead a completely normal life, without any type of physical or psychological affectation. But, as it is a pathology that tends to exacerbate with age, an early assessment by a surgeon is very important so that the deformity does not compromise the organs of the thoracic cavity or there is no significant psychological involvement.
It enables the observation of the sternal collapse in the lateral projection and the measurement of the AP diameter, as well as the transverse one. A metallic or barium marker can be placed at the bottom of the deepest part to improve visualization.
It also enables the appreciation of associated malformations, such as scoliosis, hemivertebrae, costal fusions, etc.
Patients will undergo a computed tomography scan (CT Scan or CAT) of the chest. Conventional cuts at the level of the maximum subsidence allow us to appreciate the degree of Pectus Excavatum from the calculation of the Haller Index.
It is recommended that this index be equal to or greater than 3.2, although this index should not be the only or the main value for the diagnosis of PE. Other indices should be assessed, such as the Correction Index, 3D external scan.
In the case of children, it is advisable to perform a Magnetic Resonance Imaging (MRI).
Pathophysiology of Pectus Excavatum
Approximately half of the cases affected by Pectus Excavatum will present clinical symptoms that will be due to the pathophysiological alteration of the malformation. Therefore, there will be a large population of patients with Pectus Excavatum who will not probably have symptoms or, consequently, criteria for correction.
In affected cases, the shortening of the anteroposterior diameter of the thorax causes a deviation of the mediastinal viscera, generally towards the left hemithorax, which leads to a deviation of the cardiac axis to the left and a restrictive ventilatory pattern.
In these cases, a deficit in blood filling in the right chambers of the heart can also be detected; cardiac displacement/rotation with a consequent decrease in left ventricular stroke volume, and reduced ability to pump efficiently; mitral valve prolapse; arrhythmias; or ventricular hypertrophy due to axis deviation.
Symptoms of Pectus Excavatum
The presentation of the deformity is visual and is already perceived in the first year of life, although it goes unnoticed due to the absence of symptoms, which will become evident in the prepubertal age. Cases detected early should be followed up at least until that age, since this is when the deficiencies produced by PE become apparent.
Patients suffering from this deformity may experience:
- Lack of adaptation to physical activity
The deviation of the mediastinum to the left side leads to a decrease in cardiac volume and, consequently, a lack of adaptation to physical activity.
- Respiratory problems
Decreased exercise tolerance, tiredness and shortness of breath, caused by decreased lung function; also severe restrictions of pulmonary ventilation with a restrictive pattern.
- Cardiac disorders
Precordial pain, palpitations, syncope and arrhythmias may appear.
- Morphological involvement of the spine.
It is common for the Pectus Excavatum to be associated with scoliosis, which can cause chest or lumbar pain.
Scoliosis occurs in all cases that have a certain degree of sternal rotation, which causes the ribs to rotate the vertebrae. This leads to the deformation of the spine, and not the other way around. All patients are born with Pectus Excavatum but without scoliosis. This deformity appears over time in asymmetric cases.
- Psychological disorders
Many people affected by chest deformity have behavioral changes, with a tendency to loneliness and social withdrawal.
Many cases have a mild sequence of Poland with pectoral hypoplasia of the breast and the nipple, usually the right one, which exacerbates over time and becomes more acute at puberty, especially in female patients.