Pectus carinatum is a pathology in which the front part of the thorax protrudes, taking the form of a boat keel. It is a congenital anomaly, inherited, manifested as the patient grows. It is manifested by visible deformation of the anterior part of the chest (ribs and sternum). By itself, it does not have a negative effect on the work of internal organs, but it may be accompanied by a narrowing of the chest. To clarify the diagnosis, determine the type and degree of deformation, radiography and CT are performed. Conservative treatment is ineffective. Surgical operations are performed to eliminate the cosmetic defect.
ICD 10
Q67.7 Pectus carinatum
Meaning
Pectus carinatum (keel chest) is the second most common congenital deformity of the chest after funnel chest. It is about 7% of the total number of violations of the shape of the anterior chest wall. Men suffer 4 times more often than women. In 26%, hereditary predisposition is detected, in 15% there is a combination with Marfan syndrome, congenital heart defects, scoliosis and other connective tissue diseases. Treatment of pectus carinatumt is carried out by thoracic surgeons, and in small settlements that do not have thoracic centers – traumatologists and orthopedists.
Pectus carinatum causes
Pectus carinatum is a hereditary congenital malformation. In some cases, joint inheritance of pectus carinatumt is revealed, so some researchers assume a common nature and mechanism of development of these anomalies. In addition, a reliable relationship was established between the presence of pectus carinatum, the physique of patients and the condition of their connective tissue. In most cases, patients with a pectus carinatumt have a high height and asthenic physique.
In some patients, chest pathology is combined with Marfan syndrome, a systemic disease caused by connective tissue pathology and including dolichostenomyelia (high growth), arachnodactyly (elongated spider fingers), underdevelopment of fatty tissue, hypermobility of joints, disorders of the cardiovascular system and visual organs. It is also possible to combine with congenital heart defects and scoliotic deformity of the spine.
Pathogenesis
At the birth of a child, an anomaly of the chest is usually hardly noticeable, but as the deformity progresses, and over time a cosmetic defect is formed, the severity of which can vary greatly. Functional disorders on the part of the respiratory and circulatory organs in the first years of life are not pronounced. Some older children complain of shortness of breath, fatigue and palpitations during intense physical exertion. At the same time, objective violations may be detected: an increase in the minute volume of respiration, a decrease in the oxygen consumption coefficient and the vital capacity of the lungs.
These disorders are usually caused not by the deformity itself, but by concomitant features (asthenic body type, narrowed chest, congenital heart defects). Currently, most experts believe that the keel chest itself does not entail negative consequences in the form of a malfunction of the heart and lungs and is a purely cosmetic defect.
Classification
There are a large number of classifications of pectus carinatum, but the most complete and practically significant is the version of Fokin and Bairov:
- Сostal type. The curvature of the sternum is absent or weakly pronounced and has a rotational character. The deformation is formed due to the bending of the costal cartilages anteriorly.
- Manubriocostal type. The handle of the sternum, together with 2-3 articulating costal cartilages, is curved anteriorly, and the body of the sternum with the xiphoid process is shifted posteriorly.
- Corpocostal type. There are two possible options. The first is that the sternum is arched forward in the lower and middle third, the costal cartilages are curved inwards. The second is that the sternum is obliquely directed forward and downwards and is maximally protruding in the lower third.
Both symmetrical and asymmetric deformation can be observed. In the second case, due to the improper development of the ribs, the sternum is curved along the axis.
Pectus carinatum symptoms
The shape of the chest is broken due to the sternum protruding forward, the front part of the chest will stand forward. In most patients, the cartilaginous parts of the IV-VIII ribs sink in from one or two sides. Usually, a characteristic reversal of the edges of the costal arches is revealed. The thorax is significantly enlarged in the anteroposterior direction, changes in the anteroposterior size during respiratory excursions are reduced compared to the norm or practically absent. From the outside, it looks as if the chest is constantly in a state of inspiration.
Diagnostics
The diagnosis is made by a thoracic surgeon based on the examination data, to clarify the type of deformation and the severity of changes, a chest X-ray in a lateral projection and computed tomography are prescribed. If pathology from the heart and lungs is suspected, the necessary studies are carried out: spirography, ECG, EchoCG, etc., consultations of a cardiologist and a pulmonologist are appointed. With Marfan syndrome, a comprehensive examination is indicated, including consultations with an orthopedist, cardiologist, pulmonologist, ophthalmologist and neurologist.
Pectus carinatum treatment
Among people far from medicine, it is widely believed that the pectus carinatumt can be corrected with the help of physical therapy, respiratory gymnastics and physical exercises. Unfortunately, experts have a different opinion – it is possible to restore the normal shape of the chest only with the use of surgical techniques. All other methods only improve the physical form of the patient (which is also important, especially with severe asthenia, the presence of connective tissue diseases and pathology of the cardiovascular system).
Proceeding from the above, as well as from the fact that the pectus carinatumt is mostly a purely cosmetic defect, it is clear that the optimal result in the treatment of this pathology can be achieved only by determining the priorities that are most important for a particular patient. With the same type and degree of deformation, it will be more important for one patient to restore a normal psychological background and self–esteem by performing an operation, for another – to improve the general condition of the body by prescribing a course of physical therapy and restorative treatment.
Since, unlike funnel-shaped, pectus carinatumtt does not have a clear negative effect on the functioning of the patient’s organs and systems, the only indication for surgical correction is the urgent need of the patient to eliminate the cosmetic defect. At the same time, doctors recommend resorting to surgical treatment only in extreme cases. There are two main surgical methods for the treatment of the pectus carinatumt: minimally invasive intervention by the Abramson method and open-access surgery by the Mark Ravich method.
During surgical intervention according to Ravich, the doctor performs a transverse incision under the mammary glands / pectoral muscles and cuts off the pectoral muscles and rectus abdominis from the attachment points. Then resects the costal cartilage and stitches the remaining suprachondria, reducing the intercostal spaces and bringing the sternum to a physiological position. With pronounced deformation, a wedge-shaped sternotomy is additionally performed.
Less often, open surgical interventions are used by the Kondrashin method (transverse sternotomy with resection of the deformation area and displacement of the ribs), as well as metallosternochondroplasty by Tymoshchenko. Currently, along with the listed traditional methods, minimally invasive Abramson surgery is becoming increasingly popular. When using this technique, the doctor makes two incisions 3-4 cm long on the sides, stitches plates to the ribs and attaches another straightening plate to these plates. The metal structures are removed after a few years, after complete correction of the keel deformity and reshaping of the chest.
If there are contraindications to surgery, as well as in cases when the patient is dissatisfied with the appearance of his chest, but does not agree to the listed surgical interventions, methods are offered that allow correcting the aesthetic perception of the body without correcting the shape of the chest. Men are recommended to pump up the pectoral muscles, and women are recommended to install silicone breast implants. This does not eliminate the deformation, but makes it less noticeable.
Literature
- Reactive pectus carinatum in patients treated for pectus excavatum. Swanson JW, Colombani PM. J Pediatr Surg. 2008 Aug;43(8):1468-73. link
- Surgical correction of pectus excavatum and carinatum. Robicsek F, Fokin A. J Cardiovasc Surg (Torino). 1999 Oct;40(5):725-31. link
- Chondro-manubrial deformity and bifid rib, rare variations seen in pectus carinatum: a radiological finding. Allwyn Joshua S, Shetty L, Pare VS, Sebastian R. Surg Radiol Anat. 2013 Jul;35(5):443-7. link
- Surgical correction of pectus excavatum and carinatum. Singh SV. Thorax. 1980 Sep;35(9):700-2. link