Pectus excavatum is a stuffy chest known as congenital chest wall deformity in which several ribs and the sternum grow abnormally, producing a concave or excavated part of the chest wall above.
Pectus excavatum is the most common congenital chest wall deformity, 90%, followed by pectus carinatum, cleft sternum, his PENTALOG Cnatrell, asphyxiating thoracic dystrophy and dysplasia spondilotoracica. Pectus excavatum occurs in 1 in 300 births, with male predominance. The condition is present at birth and over 90% of cases are diagnosed in the first year of life.
Worsening chest deformity and symptomatic onset of the condition are reported rapid bone growth during childhood. Many patients are not brought to the pediatrician by his parents until he adolescence. Peiptului psychological aspect can affect self-image of the young patient.
Sometimes deformation is considered only a cosmetic problem, however, depending on the severity can affect cardiac function and cause chest pain and breathing back. Mark this condition is clogged chest. The heart may be pushed or rotated, propalpsul mitral valve is often low and the capacity of the lung.
Researchers are not sure of the cause but suspected a genetic defect deformation. Approximately 37% of patients with pectus excavatum have a first degree family member with this condition.Physiology, the increased pressure from the uterus, rickets and increased traction on the sternum because of diaphragmatic abnormalities were indicated as specific mechanisms. Pectus excavatum is also a relatively common symptom of Marfan syndrome. Many children with pectus excavatum develop spinal muscular atrophy due to diaphragmatic breathing that develops in this disease.
Because the heart is located behind the sternum, and these patients have abnormalities of the heart visible damage was presumed cardiovascular function in these patients. While some studies have shown decreased cardiovascular function in pectus excavatum, there is still a consensus based on ultrasound tests on the presence of a degree of impairment of cardiovascular function in people with this condition. As there is no consensus on the degree of functional improvement after correction of the defect.
Treatment for the condition may involve invasive or noninvasive techniques or a combination of both. Before surgery is performed several tests. These include a CT scan, pulmonary testing and exams fucntiei cardiology: auscultation and electrocardiography.After the CT scan to measure the Haller index, calculated by obtaining the fraction of transverse and anteroposterior diameter of the thoracic cage. An index above 3. 25 is considered severe.Cardiopumonare tests are used to determine lung capacity and to identify heart murmurs.Corrections surgical repair of functional symptoms that may occur in this disease, such as respiratory problems or heart murmurs.
PathogenesisIn pectus excavatum cartilage and bone development is abnormal chest wall above, affecting 4-5 ribs on each side of the sternum.Defect appearance varies widely from slightly modified from severe cases of clogging up the significant asymmetry between right and left half of the thorax. Excat mechanism involved in this anomaly is not known. Not yet identified any genetic defect responsible for the development of pectus excavatum directly.Despite the lack of a genetic defect yet identified, family crowding of cases is reported in 35% of patients. The condition is associated with Marfan syndrome and Poland syndrome.
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