PECTUS ESCAVATUS

AREA DEL SITO WWW.CHIRURGIATORACICA.ORG DEL DOTT. MARCELLO COSTA ANGELI
Noi aderiamo ai principi della carta HONcode della Fondazione Health On the Net   Home DISCLAIMER   

 

Su
CARCINOMA DELLA MAMMELLA MASCHILE
PECTUS ESCAVATUS

Surgical Corrective Procedure for
Pectus Excavatum and Pectus Carinatum

Pectus Excavatum
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 pushes also the midline structures so that the lungs are compressed from side to side and the heart (right ventricle) is compressed.

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 symptomotology 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, easily fatiguability and decreased stamina and endurance become apparent especially during competitive athletics. Also, adults in their twenty and thirty year age group 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 fatiguability and cardiac arrhythmias and tachycardias during these extensive physical and strenuous exercises.

The moderately to very severe defects, where the heart is displaced to the left of the sternum or the midline, places undue pressure on the lung artery or pulmonary artery which carries blood from the heart to the lungs. This 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 also and contributes to the fatigue in contrast to the normal individual.

It is interesting, also, that there is an increased incidents in respiratory infections and asthma. Almost all of these individuals have a body configuration is that of rounded shoulders and a "potbelly". A front and lateral view x-rays of the chest 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 catherizations 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 the group at other institutions as well as our own. Studies using a bikergometer both before and after pectus excavatum chest deformity surgery has 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 because of the very young patients that are frequently asymptomatic because they are in not full exercise routines. The defect has thus remained for long periods of time into the teenage and later age group periods before the real symptomotology expresses itself. The ideal age for correction of this defect is anytime after 2 years of age and the simplicity of the repair in this age group makes this the ideal period for repair. The risk of anesthesia is minimal and also the psychologic problems in children are avoided.

The standard pectus repair of these deformities has been to operate in both chests, put a plate behind the sternum and then go through a second operation which one removes the plate after a year or so following the initial repair. This operation is quite morbid. It takes anywhere from 3-5 hours and requires 4-5 days of hospitalization. Over the past 25 years, we have designed a new operation which does not violate the chest and is combined with a bracing technique. This operation can be carried out in children between 45 minutes and an hour and in adults between 1 and 1 hour and 15 minutes and only requires 1 day hospitalization. The cost saving is one half, because hospitalization time is less.

 

Preoperative Pectus Excavatum Repair

Postoperative Pectus Excavatum Repair

Postoperative Pectus Excavatum Repair with Brace

Preoperative pectus excavatum surgery

Postoperative pectus excavatum surgery

Postoperative pectus excavatum surgery with brace

 

The incision is a bilateral 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 6-12 weeks depending on severity. During that period of time, the cartilages reform in the new position and the defect, thus, is completely corrected. The wedge osteotomy is sutured appropriately. 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 8-12 weeks after which individuals can return to their normal activities. Recurrence is very unusual with this operation because of the wedge osteotomy and the holding of the position by the wire and vest.

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

Postoperative Pectus Carinatum Repair

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, also, the respirations are inefficient and the individual needs to use the diaphragm and accessory muscles for respiration rather than normal chest muscles during strenuous exercise. Here the heart is in normal position and there is rarely a murmur. There is however, loss of pulmonary function in these individuals also and they tend to develop emphysema and also have lung infections. If one takes a careful history from the individual, they have difficulty stress exercises, walking upstairs and also asthma is increased in these individuals. 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 that also of removing the cartilages bilaterally and the excess cartilage over the sternum and 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 day and the surgical correction time is approximately 1 hour.

What are the average results of surgical corrective treatment?

Results
The results of these operations have been very remunerating as far as the ability of these individuals 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 means, 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 the 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 systems disappear.

The repairing of the chest deformity, in almost all instances, should allow the individuals the ability to participate in full activities. We have carried out over 900 of these procedures.

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).

 

*There are risks associated with all invasive surgeries. Please ask your physician to explain these risks during your preoperative consultation.

 

 

Su