Chest wall malformations have a relatively high incidence, and unfortunately, they can alter the quality of a child's life by causing both physical and psychosocial problems. Apart from pain and potential compression of important structures within the thoracic cavity (blood vessels, nerves, organs), chest wall deformities are frequently associated with cosmetic concerns and aversion to going out in public. For children with these deformities, changing for physical education class or visiting the beach can be accompanied by embarrassment, as they appear different from other children their age. And historically, chest wall deformities are misdiagnosed and neglected by physicians, leading to mistakes in the diagnosis and management. Fortunately though, over the last few decades, surgical treatment of these conditions has improved significantly, and there are now many options for suffering children. These malformations make up only a small percentage of the conditions we help sponsor, but that makes them no less significant. Please feel free to read about them in detail below and comment with any questions!
Introduction to Chest Wall Malformations
There are four major classifications based on whether the malformation is caused by cartilage, bone, or a combination of the two
Malformations may be present at birth or can develop after the start of puberty
The etiology of these deformities is not always clear. However, very often these children have associated vitamin and mineral deficiencies or connective tissue disorders like Ehlers-Danlos and Marfan syndromes
Serious physical complications are not necessarily a common association, but pathologies such as scoliosis, mitral valve prolapse, congenital hernias, airway obstruction, and superior vena cava compression have been known to occur
When children do get symptoms, usually it manifests as shortness of breath, fatigue, chest pains, and palpitations
Pictured below are examples of what these malformations may look like, as depicted in the Malformations of the Chest Wall chapter of Pediatric Thoracic Surgery
Specific Types of Chest Wall Malformations
Pectus excavatum
Also known as sunken chest
90% of cases of congenital chest wall deformities and the most common chest wall malformation that we help sponsor for treatment
More common in males than females with a 4-6:1 ratio
Caused by abnormal flexibility of the sternum, overgrowth of the ribs, and developmental failure of the bony thorax
The sternum can press inward, moving the heart to the left, compressing the right side of the heart, and reducing cardiac performance by about 20%
Most are asymptomatic but severe cases, which we pay to treat, can cause exercise intolerance, lack of endurance, shortness of breath, syncope, asthma, and recurrent upper respiratory infections
Not usually life-threatening usually but correcting significantly improves quality of life
To correct, one bar or multiple bars are inserted into the chest cavity at hinge points to keep the sternum from pressing inwards on the heart, and eventually, the shape of the chest molds into a new form that no longer places pressure on the chest. The bar is typically removed 3 years later. Pictured below, as represented in the Thoracic Wall chapter of Thoracic Surgery in Children and Adolescents
Pectus carinatum
This is a condition in which the sternum and ribs push outwards
Not usually a life-threatening condition and patients are often not symptomatic
However, the psychosocial symptoms are frequent and often severe
Pictured below are a variety of pectus carinatum cases, as borrowed from the Malformations of the Chest Wall chapter of Pediatric Thoracic Surgery
Total sternal cleft
Post-sternotomy deformity
Concluding Thoughts
This was by no means an exhaustive list. Many more types exist that are beyond the scope of this blog post. However, we were hoping to give you a glimpse into the most common types that we help sponsor for treatment. As you can probably see, these malformations have significant impacts in the quality of life in both physical and psychosocial ways.
As an organization, our primary objective is to see all Vietnamese children and their families in need receive life-saving cardiovascular surgery. We can't do this without your help! If you feel compelled to give, please click to button below. However, even if you are not able to offer a monetary contribution, you can help us by sharing our message or volunteering your time to serve with us. For those of you that already have, thank you immensely!
Stay tuned for the final piece in our series, Conditions We Treat. As always, feel free to leave questions or comments below! Best, Nick.
REFERENCES
Lima Mario, ed.Pediatric Thoracic Surgery. 1st ed. 2013. Springer Milan; 2013. doi:10.1007/978-88-470-5202-4
Petersen C, Ure B, eds. Thoracic Surgery in Children and Adolescents. De Gruyter; 2017. doi:10.1515/9783110419825