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Re: Pectus Excavatum

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Dear Elinor,

About the pectus excavatum, are you going to request he have surgery? My

son, 16, has this also and it is very noticeable. Have you had any doc's

opinions on this? In the spring we are going to go to the MDA to get another

opinion.

Thanks

Heidi mother of 5

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Dear Heidi,

His pectus excavatum is very minor, so we never considered surgery. His

pediatrician sent him to a pediatric cardiologist to see if he had Marfan's

because he was taller than us and thin, but this was before my husband's

diagnosis, so no one associated it with CMT. He is no longer thin, having

gained too much in college, and his chest hair covers the pectus. His calves

are well defined and his arches normal.

Elinor

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Ok, pardon my ignorance but what is a pectus excavatum?

**************************************

http://www.pectusdeformity.com/

The defect known as pectus excavatum, or funnel chest, and pectus carinatum,

know as pigeon breast, are congenital anomalies of the anterior chest wall. The

excavatum defect is characterized by a deep depression of the sternum, usually

involving the lower half or two thirds of the sternum, with the most recessed or

deepest area at the junction of the chest and the abdomen. The lower 4-6 costal

or rib cartilages, dip backward abnormally to increase the deformity or

depression and push the sternum posterior or backward toward the spine. Also, in

many of these deformities, the sternum is asymmetric or it courses to the right

or left in this depression. In most instances, the depression is on the right

side. Also, because of the pressure of the sternum and cartilages, the abdomen

looks like a " potbelly " . The entire defect also pushes the midline structures so

that the lungs are compressed from side to side and the heart (right ventricle)

is compressed and displaced.

The pectus excavatum defect is found in somewhere between 1 in every 500-1000

children. It does occur in families and thus, is inherited in many instances.

Other problems, especially in the muscle and skeletal system, also may accompany

this defect. In approximately 1/5 of the patients, scoliosis is present. The

defect is seen shortly after birth and then progresses to its maximum after the

growth period in adolescence. The regression or any improvement in this defect

rarely occurs because of the fixation of the cartilages and the ligaments. When

one takes a deep breath or inspires, the defect is usually accentuated.

More at website

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Hi Elinor,

Thanks for your input. We will definitely look more into his PE. I hope

the MDA will have some answers. It is a process with CMT - all the doc's

appointments and waiting for answers for this and that. My son has gone through

two major hip surgeries one at 3 1/2 and one at 9. So far so good--does not

need that again. It is stressful to the family the surgeries--especially leaving

the other children at home while we go through the surgery. I empathasize with

all that have been through this.

Again, thanks.

Heidi

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