Pectus Excavatum and Minimally Invasive Surgery

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Pectus Excavatum and Minimally Invasive Surgery




Guest:  Dr. Andre Hebra - Surgery

Host:  Dr. Linda Austin – Psychiatry


Dr. Linda Austin:  I’m Dr. Linda Austin.  I’m interviewing Dr. Andre Hebra who is Professor of Pediatrics and Surgery and Director of Pediatric Surgery here at the Medical University of South Carolina.  He is an internationally known expert in the surgical repair of a condition called Pectus Excavatum.  Dr. Hebra, could you explain what that is, please?


Dr. Andre Hebra:  Pectus Excavatum is a congenital malformation of the chest in which the sternum and part of the ribs in front of the chest are caved in, which gives a variety of problems and it looks very bad. 


Dr. Linda Austin:  I would imagine this is something that parents can see as soon as a baby is born. 


Dr. Andre Hebra:  That’s correct.  The majority of children that have Pectus Excavatum are diagnosed early in life.  However, there are a number of kids in which the condition is very mild when they’re infants and young babies, but as they grow older, especially during puberty when they start growing fast, all of sudden the chest will change and assume this very pronounced caved-in appearance.


Dr. Linda Austin:  How common is this disorder?  I’m sure it’s a question of degree.


Dr. Andre Hebra:  Pectus is estimated to occur in about 1 in 500 to 1 in 1000 live births, so it’s relatively common.  In a specialized center like ours, we see many adolescent children that have this condition.  In this day and age, it’s something that needs to be looked at early on, as early as possible, to be followed and evaluated by an expert in the field.


Dr. Linda Austin:  Is it genetic, for example, if a parent has one child with this, should they be concerned that a second child born might also have it?


Dr. Andre Hebra:  Yes.  Unfortunately, we do not know what specific gene is involved.  But, without any doubt, there is a familial occurrence in more that 30 percent of the patients in which they will tell you that the father or grandfather, or another sibling, had it.  It is also associated with connective tissue disorders, such as marfan syndrome or poland syndrome.  It can be associated with other less common anomalies, like mitral valve prolapse, and so forth.  So, again, it is important that once you have a child that has this condition, they should be evaluated by somebody that has expertise in that area.


Dr. Linda Austin:  Now, you mentioned early on that not only is it a cosmetic problem that the child might be self-conscious about, or an adult might be self-conscious about, but it can cause health problems as well.  What are those problems?


Dr. Andre Hebra:  By far, the number one reason patients are referred to us is because of the appearance of the chest, because it’s so striking to see that sternum caved in.  A lot of adolescents have psychological problems related to the appearance of the chest.  But, the physical problems depend on the severity of the Pectus.  In very mild cases, there will be minimal physiological impairment.  However, in more severe cases, what the caved-in sternum will do is actually displace the heart to the left side of the chest and cause a rotational anomaly of the heart.  Occasionally, this can cause mitral valve and tricuspid valve regurgitation and it compresses on the lungs, decreasing, what we call, pulmonary reserve.  So, many young kids and teenagers will tell you that they have shortness of breath.  They’re unable to keep up with the peers.  They don’t perform as well in sports.  They, occasionally, will have chest pain and discomfort.  So, again, these symptoms that we see will depend a little bit on the severity of the deformity.  


Dr. Linda Austin:  At what age do you begin to think about surgical repair of a pectus?


Dr. Andre Hebra:  It used to be, with open surgery, that these patients were treated when they were older, as teenagers.  But, with the advent of the new treatment modalities, the so-called minimally invasive repair, we now like to plan surgery between the ages of 8 and 12, at a much younger age. 


Dr. Linda Austin:  Do you prefer to do it in a younger child or an older child, if there’s not a physical reason for making the repair?


Dr. Andre Hebra:  The big advantage of operating on younger children is that the operation itself is a lot easier.  The recovery from surgery is much better and the cosmetic result and the repair you can achieve is much better on somebody in which the rib and sternum are still developing.  If you intervene, say, between 8 and 10 years of age, you will get a much better result than if you’re operating at 15 years of age.


Dr. Linda Austin:  Now, your major contribution has been in the use of minimally invasive techniques for doing this.  Why is that such an advantage?


Dr. Andre Hebra:  Well, several reasons.  The open surgery, also known as the ravage operation, is a very good surgery that was developed and described in the 50s.  But it requires a large incision in the front of the chest, almost nipple to nipple, and then you have to create muscle flaps and expose the ribcage.  You actually remove the segment of the rib and cartilage that is affected by this deformity and you have to fracture the sternum in several places to make it flat again.  And you have to sort of reconstruct the anterior chest wall.  It works, but it leaves a scar.  It takes several hours to do.  It’s a very painful operation.  It’s associated with blood loss.  You frequently need chest tubes, and so forth.  So, the popularity of the open repair has never been very good.  


About 10 years ago, a new technique came about, the so-called minimally invasive repair.  It’s also referred to as the Nuss operation, because Dr. Nuss, in Virginia, was the pioneer in developing this new concept.  He realized that you can correct this deformity by placing a stainless steel bar behind the sternum using minimal access surgery.  In other words, all you’d do is two small incisions on the side of the chest and then place this bar that will push the sternum and the rib cage out and remodel the ribcage and the sternum, similar in fashion to braces for your teeth.  The difference is, with the operation, you achieve an immediate result of how the chest will look. 


A few years later, we actually modified the operation a little bit to make it better and safer for the patients and achieve better results by introducing thoracoscopy.  So now the operation is done with minimal access surgery and thoracoscopy, which means there’s a small camera inside the chest that allows us to look and see where the bar goes, place the bar very precisely where it needs to be and secure the bar in such a way that it will not move.  One of the problems with the early onset of the operation is that the bar would actually move somewhat.  Nowadays, with the modifications to the technique that we developed, the bar is unlikely to move and the results are much better.


Dr. Linda Austin:  Boy, that’s very exciting.  Now, does the bar stay there, then, for the rest of that person’s life, or is it ever removed?  What happens after the surgery?


Dr. Andre Hebra:  Typically, the bar will stay for about two years.  Anywhere between two and three years is the recommended length of time for keeping the bar.  After that time, the ribcage will have remodeled itself so that the bar can be removed and it [the ribcage] should stay the way it’s supposed to be.


Dr. Linda Austin:  What are some of the possible adverse consequences of this procedure?  When there are problems, what kinds of problems might there be?


Dr. Andre Hebra:  The procedure itself has changed from what it used to be, about 10 years ago, to what it is today.  The early reports and one of the large outcomes study, published by Dr. Nuss and I, looked at the long-term outcomes of this procedure in the early series, before the use of thoracoscopy and before the use of modern ways of stabilizing the bar, and the complication rate was relatively high.  It was about 10 to 20 percent, which is very high for any type of surgery. 


But, with the modifications that we introduced to this technique and the new ways of securing the bar and the new ways of looking at the bar placement, the complication rate has dropped to less than five percent.  So, nowadays, we consider the operation relatively safe.  Some years ago, there was some reservation as to whether this operation would be recommended for everybody.  But, nowadays, we can say that the advantages of this approach, leaving essentially minimal scars on the side of the chest that are not even visible and correcting this deformity is really the way to go.


Dr. Linda Austin:  So, what percentage, then, of patients who come to you end up getting this technique, as opposed to the old open procedure?


Dr. Andre Hebra:  I can safely say that I haven’t done the old open approach in more than six years. 


Dr. Linda Austin:  That’s really something.  Now, in that less than five percent where there are complications, what are those sorts of complications?


Dr. Andre Hebra:  The number one thing that we worry about is the risk of the bar displacing or moving.  What that means is, if the patients are not totally compliant with our postoperative recommendations, if they become too active too early, they run the risk that the bar is still not quite fixed enough to where it needs to be and it may move up or down or sideways.  Usually, if there’s a little movement of the bar, it’s not a big deal.  However, if the displacement is more than 90 degrees, it may require a reoperation.  So, we counsel our patients.  We work with them very closely so that they know exactly what they can do and what they shouldn’t do.  One example is they should avoid contact sports.  Contact sports are not allowed during the time the bar is in place because of the risk of displacement.


Dr. Linda Austin:  Dr. Hebra, thanks so much for talking with us today.


Dr. Andre Hebra:  Thank you.


 If you have any questions about the services or programs offered at the Medical University of South Carolina or if you would like to schedule an appointment with one of our physicians, please call MUSC Health Connection:  (843) 792-1414.


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