What is Pectus Carinatum?
Pectus Carinatum is an extremely rare deformity involving the chest wall in which the breastbone pushes outward instead of being against it. It is also termed as Pigeon Chest or Keel Chest. Anatomically speaking, during development of the chest wall the cartilage that connects the ribs grows flat along the chest; however in a person with Pectus Carinatum there is abnormal growth of the cartilage. This results in abnormal growth of the chest wall in the area where the ribs connects to the sternum. This is the reason why the chest wall appears outwards.
Pectus Carinatum in most cases occurs during childhood and becomes more and more observable as the child attains puberty. Inn instances, Pectus Carinatum may not be evident until a child goes through a growth spurt.
What Causes Pectus Carinatum?
As stated, the primary cause for Pectus Carinatum is the abnormal growth of cartilages that connect the ribs to the sternum which causes the chest wall to push outward. Why does this abnormality occur is still a matter of ongoing research. There are some studies that suggest it tends to run in families pointing towards a genetic link. Pectus Carinatum is seen more in people with Marfan syndrome, Noonan syndrome, Ehlers-Danlos syndrome, and certain chromosomal abnormalities.
What are the Symptoms of Pectus Carinatum?
The primary presenting feature for Pectus Carinatum is outward pushing of the breastbone and this is the only symptom that an individual has. There are instances where the person may also complain of shortness of breath with exertion, tenderness around the breastbone area, asthma, fatigue, tachycardia, and frequent bouts of respiratory infections.
Some children have a condition called pectus excavatum in which there is worsening of the Pectus Carinatum on one side and the other side tends to have a dip. It is this dip that is termed as pectus excavatum. Pectus Carinatum is also at times associated with cardiac conditions, scoliosis, kyphosis, certain connective tissue disorders, and certain musculoskeletal abnormalities.
How is Pectus Carinatum Diagnosed?
If an individual is suspected to have Pectus Carinatum then a radiographic study in the form of x-rays will be done to check the status of the growth of the breastbone. PFTS may also be done to check whether the lungs are functioning normally and see if Pectus Carinatum is causing any respiratory infections.
For people with cardiac symptoms will have to undergo EKG. CT and MRI scan is also done in some cases to arrive at a diagnosis of Pectus Carinatum. The physician may also conduct certain additional tests to look into the possibility of underlying conditions like scoliosis and Marfan syndrome which is quite common with Pectus Carinatum.
How is Pectus Carinatum Treated?
Pectus Carinatum is more of a cosmetic disorder where the appearance of the chest wall is abnormal even though some people experience certain additional symptoms like difficulty breathing with exertion or frequent respiratory infections due to it. The treatment for Pectus Carinatum is based on how bad the appearance of the chest wall is as well as the symptoms that the patient has associated with the condition.
Bracing is the first treatment that a patient with Pectus Carinatum gets, especially during childhood and adolescence. This is done when the condition is mild or moderate at most. A custom fit brace is made and the child has to wear it on the chest. The brace puts pressure on the area that is most affected by Pectus Carinatum to make it more flat. The brace will have to be worn for at least eight hours every day for a few months until improvement is seen. Sometimes, they have to wear it till the child attains puberty.
Surgery is the next mode of treatment for Pectus Carinatum. This is done when bracing is deemed ineffective or the child has attained puberty. Surgery is also recommended for people where the symptoms of Pectus Carinatum are extremely severe. The surgery done to correct this deformity is known as Ravitch procedure. This procedure involves removal of abnormal cartilage and replacing the breastbone back into normal position.
The surgeon may also opt to break the breastbone to place it back into the normal position. In some people, a metal chest bar is placed temporarily and connected to the ribs with wires. With regard to the overall prognosis, Pectus Carinatum does not affect the health of a child. Normally, bracing is quite effective in treatment of Pectus Carinatum.