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Surgical Corrective Procedure for Pectus Excavatum and Pectus Carinatum
 
Correction of chest wall defects known as pectus excavatum (sternal depression) and pectus carinatum (sternal protrusion) are now available to patients. At the University of Minnesota, Pediatric Surgeons, Robert Acton MD and Daniel Saltzman MD Ph.D., perform both major techniques of pectus repair (Ravitch and Nuss) as well as the Leonard Technique for the Pectus repair. Dr. Arnold Leonard, now retired Adjunct Professor of Surgery from the University of Minnesota, has performed more than 1500 of these procedures with remarkable success. Upon his retirement, he has "passed the torch" to his partners.

  What is Pectus Excavatum and Pectus Carinatum?

Pectus Exacavatum
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.

  What are the symptoms related to pectus excavatum and pectus carinatum?

The symptomatology of this defect many times is not really apparent until the child participates in athletic or high stress activities. As children, the individuals are shy and many times will not participate where their chest is exposed such as in swimming or athletic events. As the child progresses in age, the symptoms, easy fatigability, decreased stamina and decreased endurance become apparent especially during competitive athletics. Also, adults in the twenties and thirties year age groups and elderly adults, become very aware of this problem. If they have not participated in physical activities during high school, newer fitness programs bring out the easy fatigability; cardiac arrhythmias and tachycardia during these extensive physical and strenuous exercises have been reported. The moderately to very severe defects, where the heart is displaced to the left of the sternum or the midline, may cause a murmur and this murmur is really due to the pressure on the system causing rough instead of smooth flow. The electrocardiogram also can demonstrate strain on the right side of the heart. The expansion of the lungs during breathing or exercise which is important to maintaining normal respiratory function is confined because the chest wall cannot expand. Thus, more intensive and rapid respiratory rate is necessary. Also, the diaphragm is called upon to make larger movements to provide enough oxygen and carbon dioxide exchange to meet the demand of the body under exercise conditions. More energy is thus utilized for breathing and contributes to fatigue, in contrast to the normal individual.

It is interesting, also, that there has been an increased incidence in respiratory infections and asthma reported by several authors. Almost all of these individuals have a body configuration of rounded shoulders and a "potbelly". Front and lateral view chest x-rays demonstrate the defect and displacement of the heart to the left of the midline as well as compression of the right ventricle. How can one test this defect with pulmonary function test or cardiac output evaluation? In most instances, conventional pulmonary function tests or cardiac stress echocardiograms to measure cardiac output and function are normal with the patient at rest. If the patients, however, are subjected to upright intense exercise, the cardiac output is usually decreased when compared to normal individuals of the same age. Also, the respiratory function is reduced, and depending on the severity of the defect, this reduction can be from 10-30%.

After correction of the defects, the function returns to near normal in the majority of the patients. These studies have been corroborated by groups at other institutions as well as our own. Studies using a bike-ergometer, both before and after pectus excavatum chest deformity surgery, have shown an increased ability to expand the lung and exchange oxygen and carbon dioxide. Also, fatigue rarely occurred after expansion of the lung. Also, the heart rate is slower and the ability to exercise at high levels of energy output is improved. It is important, therefore, to realize that the pectus excavatum deformity is not just a cosmetic problem. One only has to ask the 30 and 40 year old or the athletic teenager whether he is having difficulty with his strenuous exercise activities. A good history and physical exam is important. This history combined with the x-rays should be the indication for surgical intervention. Patients with moderately to severe uncorrected deformity usually cannot be competitive in major activities. The continuous beating of the heart against a firm bone may also lead to arrhythmias. We have treated patients up to 70 years of age.

  What can be done to correct these conditions?

Surgical Corrective Treatment
     

It is unfortunate that this defect has been deemed cosmetic over a long period of time. Primarily because of the fact that very young patients are frequently asymptomatic. The defect has thus remained for long periods of time into the teenage and later age group periods before the real symptomatology expresses itself. The ideal age for correction of this defect is between 12-18 years of age and the simplicity of the repair in this age group makes this the ideal period for repair.

The standard pectus repair (Ravtich approach) of these deformities has been to place a sternal bar (Adkin's Strut) behind the sternum after mobilizing the deformed cartilages around the sternum and then go through a second operation to remove the bar after approximately 12 months following the initial repair. The Nuss Procedure involves the placement of a large curved bar through incisions on the chest wall. The bar is rotated into position and kept in place for 2 to 3 years.

     
Preoperative pectus
excavatum surgery
Postoperative pectus
excavatum surgery
Preoperative pectus
excavatum surgery with brace


The incision is a transverse curvilinear incision beneath the breasts, which gives a good cosmetic scar. The lower 4-5 cartilages are removed and the perichondrium or the covering of the cartilages is left in place. Then a wedge osteotomy or wedge is taken out of the sternum and depending on whether there is asymmetry the sternum is tailored obliquely according to the defect. A sheathed wire then is placed behind the sternum and then brought out through the muscles and skin and later attached to a modified brace for a period of 12-15 weeks depending on severity. During that period of time, the cartilages reform in the new position and the defect, thus, is corrected. The patients are fit with a brace prior to surgery which is a light vest to which the wire is attached at surgery. Patients can return to work within a week after surgery and children may go back to school within that period of time. Blood administration is unnecessary. The complete healing period is 3 months after which individuals can return to their normal activities. Recurrence is very unusual with this operation.


Pectus Carinatum
Pectus carinatum, or protrusion of the breast (pigeon breast), is an entirely different malformation. The overgrowth of the cartilage and forward buckling onto the sternum and secondary pressures cause pain to be present. In most instances, the peak progression of this defect occurs during the growth periods, especially in teenagers and thus, the defect is usually corrected at this time.
Preoperative pectus
carinatum surgery
Postoperative pectus
carinatum surgery
Postoperative pectus
carinatum surgery without brace

What is important to recognize is that this carinatum deformity produces a very rigid chest so the chest is almost secured in a position near full inspiration. Thus, respirations are inefficient and the individual needs to use the diaphragm and accessory muscles for respiration rather than normal chest muscles during strenuous exercise. These finding are typical for a restrictive lung disease type pattern. Here the heart is in normal position and there is rarely a murmur. There is however, loss of pulmonary function. If one takes a careful history from the individual, they have difficulty with strenuous exercises, walking upstairs and they may have asthma. Obviously in both defects, asthma is not cured by an operation but certainly the hospitalizations and the necessity for drugs are reduced by the surgical correction. The surgical correction for this defect is to remove the affected cartilages bilaterally and the excess cartilage over the sternum and a reverse wedge is carried out on the sternum and then bracing is in a compression system rather than the outward rigging that is required by a pectus excavatum. Again, the hospitalization is 1 to 2 days and the surgical correction time is approximately 1 hour.

  What are the average results of surgical corrective treatment?

Results

When our patients were specifically asked, the results of these operations have been rated excellent and very good. Most individuals have reported an increased ability to participate in normal strenuous activities and athletics.† Also, there is a marked improvement in the patient's self image. The operations are carried out for physiologic reasons, not for cosmesis, although the cosmesis is a benefit with the correction. Many of the small children are teased by their schoolmates and they become very inhibited and reclusive. They, many times, refuse to take showers with the other kids and during physical education, refuse to take their shirts off during these activities. We have found that this does not decrease with age. We have carried out a number of procedures in 30-40 year olds and even in 60 year olds who have marked limitation in physical and social activities. In the 60 year old age group, arrhythmias have been the most challenging problem and these have been referred in by cardiologists. Once pressure on the right ventricle is relieved, the symptoms disappear.

The repairing of the chest deformity, in almost all instances, should allow the individuals the ability to participate in full activities. Because this new procedure has a marked decrease in morbidity and cost, greater numbers of patients are having this surgical correction performed (especially in the 30-40 year old age group). This new corrective procedure is rarely turned down by insurance companies due to the increased understanding of it not being a cosmetic defect.

If you would like to learn more about this procedure or have any questions, please contact us at:

Robert D Acton MD and Daniel A Saltzman MD Ph.D.
195 MMC 420 Delaware Street S.E. Minneapolis, MN 55455
Telephone: (612) 626-4214
pedsurg@umn.edu

*There are health risks associated with all invasive surgeries.


References: Wolf WM., Fischer MD, Saltzman DA, and Leonard AS:
Surgical Correction of Pectus Excavatum and Carinatum. Minnesota Medicine 70:447-453, 1987
Soto L, Kirzeder D, Larsen D, Saltzman D, Leonard A; The Leonard Modification of the Ravitch Procedure for Pectus Excavatum Repair, J Amer. Coll. Surgeons, 197(3suppl): S32, 2003.


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