How is funnel chest formed?

Written by Zhang Zhi Gong
Cardiothoracic Surgery
Updated on November 04, 2024
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Regarding the cause of pectus excavatum, there have historically been many hypotheses. For a while, medical experts believed that pectus excavatum was caused by the backward pulling of the diaphragm behind the sternum. Thus, for a time, surgeries abroad involved cutting the portion of the diaphragm behind the depressed area of the chest. Eventually, it was discovered that such cutting did not significantly benefit the treatment of pectus excavatum, and the results were not very conclusive. Therefore, this hypothesis was later debunked. To date, it is generally believed that the primary formation of pectus excavatum is somewhat related to genetic or hereditary factors. Of course, not all cases of pectus excavatum are due to parents having the condition; it might be present in the genes of ancestors and only manifest in the current generation or in this patient. Additionally, pectus excavatum could be associated with other diseases, such as connective tissue disorders. For example, some patients might have congenital diaphragmatic hernias, and after surgical repair, they could be prone to pneumothorax. Furthermore, some patients may suffer from pectus excavatum due to subglottic stenosis and underdeveloped bronchopulmonary structures, possibly triggered by respiratory factors. However, no matter the details, the formation of pectus excavatum is directly or indirectly related to congenital genes or heredity.

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Written by Zhang Zhi Gong
Cardiothoracic Surgery
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How to treat pectus excavatum?

There are many treatment methods for pectus excavatum, and the choice depends on the severity of the pectus excavatum, the age and chest wall elasticity of the child with pectus excavatum, the potential for further growth and development, and the expectations of the patient and their family. For younger children with pectus excavatum, where the chest wall is more elastic and soft, and in cases of mild pectus excavatum, a pectus excavatum suction cup can be considered. This device uses a certain amount of pressure, similar to a car suction cup, which is commonly seen in auto repair shops where a dented plastic bumper is gradually pulled out using a suction cup. This principle is also utilized by the pectus excavatum suction cup. Of course, this is under the premise that the patient is younger and has a softer chest, making it easier to be corrected by suction. For older patients with a harder chest, using a suction cup might not be appropriate, and surgical treatment may need to be considered. There are several surgical techniques available, ranging from the early Ravitch procedure, which involves a sternotomy and complete detachment of the sternum followed by flipping it, to the later Nuss procedure, and up to the current Wang surgical method and minimally invasive techniques.

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Written by Zhang Zhi Gong
Cardiothoracic Surgery
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Best age for pectus excavatum surgery

The best age for funnel chest surgery, according to the latest and most authoritative ninth edition of the surgical textbook, is between three and five years old. Historically, there has been controversy over the best age for funnel chest surgery, with some pediatricians previously believing it should wait until adolescence. However, it has been found that by the age of five, children start to become more aware and might realize their chest shape differs from others, potentially leading to feelings of inferiority and reluctance to make friends. Thus, performing the surgery before the age of five—before the child is fully aware of their deformity—might actually be preferable, as it could minimize psychological and physiological impacts. Of course, there is also a viewpoint supporting surgery before the age of three, but the younger the child, the softer the chest bone, which sometimes allows for other potential corrective methods.

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Written by Zhang Zhi Gong
Cardiothoracic Surgery
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Is pectus excavatum hereditary?

Is pectus excavatum hereditary? According to modern genetic medicine, actually, about 80% of diseases are related to genetics to some extent, and pectus excavatum is no exception. Normally, pectus excavatum occurs in about one in 400 to 1000 people, with a higher prevalence in males. Research has also found that pectus excavatum is often seen in several genetic disorders, including Noonan syndrome, Turner syndrome, and Marfan syndrome. This indicates that it shares certain genes with these genetic disorders, such as the fibrillin-1 gene and others in the RAS/MAPK pathway. These genetic correlations may not always be evident, for example, the parents may not have pectus excavatum themselves. However, when parents with these recessive genes reproduce, their combination might result in pectus excavatum in their child. The development of pectus excavatum might be related to abnormal asymmetrical development of the cartilage. Thus, there is indeed a certain correlation between pectus excavatum and genetic factors.

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Written by Zhang Zhi Gong
Cardiothoracic Surgery
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What should be noted for pectus excavatum?

We know that behind the sternum of a normal person are the heart and lungs. Pectus excavatum is a deformity where this part of the sternum is pressed inward towards the spine. Such inward compression, as it occurs right against the bony spine, will directly squeeze the heart and in severe cases, can lead to insufficiency in the heart valve closure. Therefore, the most critical issue with pectus excavatum is the compression of the heart, which also compresses the lungs. Thus, 80% of children with pectus excavatum have lower exercise capacity compared to normal children, 60% may experience chest pain, and 40% have a significantly higher respiratory rate than normal children. Therefore, for patients with pectus excavatum, it is crucial to be cautious about common colds, as their respiratory and cardiac functions can be greatly affected if they catch a cold.

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Written by Zhang Zhi Gong
Cardiothoracic Surgery
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Is pectus excavatum hereditary?

In current medical technology, although no genes exclusively associated with pectus excavatum have been identified, there is evidence suggesting genetic predisposition when pectus excavatum coincides with other conditions. For instance, the incidence of pectus excavatum is significantly higher in patients with congenital connective tissue disorders, such as Marfan Syndrome, and is linked to certain genes within this syndrome. Additionally, patients with congenital diaphragmatic hernia also show an increased incidence of pectus excavatum. Moreover, the condition is more prevalent in patients, or children, who have subglottic airway stenosis or congenital bronchopulmonary dysplasia. Thus, these evidences confirm that pectus excavatum, as a disease in itself, is directly or indirectly related to congenital genetic factors, indicating a certain hereditary nature.