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PECTUS CARINATUM


PECTUS
Pectus Carinatum most known as “Pigeon’s Brest or “keel chest” has not received the same interest as Pectus Excavatum by clinicians, pediatrists, orthopedists and pediatric surgeons. Even though since 1953, when Lester published his first surgical correction, thoracic surgeons have been concerned about the surgical solution for this deformity.

Robiseck survey among 41,863 school children found the prevalence of 0.6 / 1,000. Marlos Coelho found a prevalence of 0.97 /1,000 surveying 106,709 teenage students in Curitiba.

There is a prevalence of men over women. Most published series showed a prevalence of Pectus Excavatum over Pectus Carinatum in a range of 3:1 until 13:1. In our series, contrary to what has been shown in literature, we had a prevalence of 1:2 among operated patients and in our teenage school children of 1:2.

The defect, most of the time, progresses as the individual grows and very few or none show cardiac or respiratory symptoms. If symptoms are present, in most cases they are related to an associate disease or are from psychological order what causes the patient to be introverted, slow, non-enthusiastic, show inferiority complex and sometimes complaining about palpitations, dyspnea, thoracic pain, and lassitude. After the surgery all symptoms not related to an associated disease will disappear.

Any limitation in working, physical or sport activities should be attributed to emotional alterations produced by the deformity. These patients avoid going to swim on pools, beaches and practicing activities where the chest is shown. Even when the thorax is covered, they curve the torso forward with dropped shoulders so as to minimize the esthetic deformity.
 
1) INFERIOR PECTUS CARINATUM
- Chicken Brest, Pigeon Brest

The classic deformity is constituted by sternal prominence, especially in its medial and inferior portion and most of the time accompanied by inferior bilateral costal depression, caused by the curving of costal cartilages and ribs extremities downward. We have named of Symmetric or Classic Pectus Carinatum and Asymmetric or Lateral Pectus Carinatum because we think they have the same etiology and pathogenesis. A lateral X-ray of thorax shows the sternal protrusion forward with evident gladiomanubrial articulation and sternal sutures. Sternal tomography and computerized thoracic tomography shows the deformity and normal sternal structure. This deformity is present at birth, in a discreet form and in a small number of infants, becoming more visible in puberty because of growth sprout.

In cases of Lateral Pectus Carinatum, thoracic computerized tomography shows the obliquity of the sternum in relation to the body axis, which is very important for surgery planning.
 
INFERIOR PECTUS CARINATUM (Chicken Brest, Pigeon Brest)
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Classic Or
Symmetric
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Lateral Or
Asymmetric
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Symmetric
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Symmetric
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Asymmetric
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Asymmetric
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Symmetric
 
X - RAY
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X-Ray showing a
great sternal protrusion
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X-Ray showing a
great sternal protrusion
 
COMPUTERIZED TOMOGRAPHY
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Computerized tomography showing
asymmetric chondrosternal protrusion
 
2) SUPERIOR PECTUS CARINATUM
- Pouter Pigeon, Mixed Pectus, Chodromanubrial Protrusion with Chondrogladiolar Depression or Currarino-Silverman Syndrome

This is the less frequent form, occurring with a prevalence of 1:9 compared to Pectus Excavatum. In school children of Curitiba series its prevalence was 1:5. It is constituted of superior protrusion of manubrium and of body proximal or gladiolar and acute depression of sternum; in most cases it is an inferior pseudodepression. For some authors this would be the mixed form of Pectus Excavatum/Carinatum. A lateral thoracic X-Ray shows an arch form sternum, sometimes an S form as a single bone, smaller than expected with a complete union between sternum body and manubrium. Sternum tomography and computerized tomography show an absence of manubrium-gladiolar joint with sternal sutures obliteration and ossification of all sternal ossification nucleus. This deformity is present at birth and it does not change its aspect as the child grows. It grows proportionally to the child’s development.

Other bizarre forms may occur, which we see as variations of the ones already described above. Superior or inferior costal protrusions, uni or bilateral, are, as we see, isolated alterations of costal cartilages.
 
Superior Pectus Carinatum
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Superior
Carinatum
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Superior
Carinatum
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Superior
Carinatum
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Superior
Carinatum
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Superior
Carinatum
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Superior
Carinatum
 
X - RAY
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X-Ray evident
sternum arch form
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X-Ray evident
sternum arch form
 
ETIOPATHOLOGY
Classic Pectus Carinatum is less frequent perceived at birth than Pectus Excavatum. For that reason it is believed that its occurrence is more acquired than congenial. In most cases it is perceived around 10 years of age.

Facts favoring the congenial theory: the occurrence of two Pectus Carinatum in the same family; Superior Pectus Carinatum observed at birth in all the cases; occurrence of Pectus Carinatum associated with Marfan’s Syndrome and with congenial cardiopathies and hand agenesis.

An intimate relationship with bronchitic asthma and bronchial asthma, according to Haller, could bring about Pectus Carinatum at puberty because of intrathoracic raising pressure. Coelho mentions bronchitic asthma and chronic bronchitis occurrence in 67% of cases.

Currarino and Silverman demonstrated that the premature obliteration of sternal sutures and of the ossification nucleus is a Pectus Carinatum characteristic, being present since birth. It is congenial but not hereditary. Most of the authors consider that the exaggerated growth of cartilages involved in the deformity would push the sternum forward (Pectus Carinatum) or backwards, depressing it (Pectus Excavatum). If the growth is only unilateral or asymmetric, the protrusion would be unilateral or predominantly unilateral. Why this growth happens it is not explained. We observed that Pectus Carinatum is accentuated in adolescence.
 
TREATMENT
PHYSIOTHERAPY

It is unanimous that such treatment does not bring any improvement to this deformity. In discreet defect that are improved with growth, it may help. We recommend sports practice in general, especially swimming. Lifting weights will only accentuate the defect. The Global Posture Re-education is indicated to treat postural bad habits, kyphosis or scoliosis that may be associated to the deformity.


ORTHOPEDIC / ORTHOTIC

Literature refers the use of apparatus and belts with the aim of compressing the thorax and this way modeling the deformity. Belts and bands do not have any therapeutic effect.

In Brazil, Haje has used for a long time the Dynamic Thoracic Compressor (DTC). It applies a selective compression over the sternum, using the contrapressure of the spine. Such apparatus has advantages over belts and bands, which compress the entire thorax causing ventilation problems. The inconvenience is the daily and long time of usage. During all the day the patient must use the apparatus taking it off just to take a bath and eventually to exercise or for physiotherapy session during the first 6 months. The treatment may be as long as 2 years. The orthotic treatment is indicated according to the flexibility of anterior thoracic wall, which depends of the deformity degree and patient’s age. In Superior Pectus Carinatum and Lateral Pectus Carinatum this treatment is indicated during childhood only when the defect is to serious once the child may get tired of the apparatus and, as we know, during the growth sprout of puberty the defect is accentuated or may return. The best indication is for teenagers whose deformity is flexible and reducible.

The rates of excellent and good results for patients who finish the treatment are 68%. 24% for Inferior Pectus Carinatum, 47% and 39% for Lateral Pectus Carinatum and 9% and 21% only for Superior Pectus Carinatum. It is important to tell the family about the poor results of this treatment in this type of Pectus.

Haje does not mention the recidivism percentage after this treatment.

There is a great difficulty for children and teenagers to keep the treatment going. Only 60% of them come back to continue the treatment. Despite the patients’ reluctance to use prosthesis, this should be encouraged in the cases of Inferior and Lateral Pectus Carinatum and with restrictions in Superior Pectus Carinatum following the organization chart we proposed for the treatment of Pectus Carinatum.
 
SURGICAL TREATMENT – INDICATION
Most authors indicate surgery since the deformity is evident or grotesque.

Since it is an esthetic surgery, information about results, scar, etc, should be only given to parents.

In general, as in Pectus Excavatum, we prefer to perform surgery after 10 years of age, except for a grotesque deformity or patients with severe psychological alterations caused by the deformity.

Below we show our organization table for the assistance of patients with Pectus Carinatum.
 
ASSISTANCE ORGANIZATION CHART
 
TYPES OF SURGICAL TREATMENT
As in Pectus Excavatum literature reports several techniques based on the subperichondral resection of involved cartilages and sternal osteotomy, if necessary. Later we will describe the technique we developed for the treatment of Pectus Carinatum and Pectus Excavatum.
 
SURGICAL TECHNIQUE FOR THE TREATMENT OF PECTUS EXCAVATUM AND PECTUS CARINATUM
STERNOCHONDROPLASTY MODIFIED BY MARLOS COELHO


Principles:

1- Subperichondral resection of cartilages involved in the deformity. Since the perichondrium is the germinative element of cartilage, so it “produces” cartilage, in 3 months the costal cartilage will be reconstructed in an adequate position beginning from the maintained perichondrium.

2- We do not resect the intercostal and perichondral muscles bundles longitudinally and bilaterally, separating them form sternum to perform osteotomy/osteotomies as some authors recommend, or as a phase of the technique to be applied. As some authors do not reinsert the muscleperichondral bundles, the anterior thoracic wall becomes flabby and a palpable and/or visible parasternal “runlet”. We think the pleating of musculocatilagenous bundles resulting from subperichondral resection is essential to give stability to the anterior thoracic wall and to “pull” laterally the sternum and to rectify the bundles as to the “new cartilage” acquire the correct position.

3- We always perform the osteotomy/osteotomies in the anterior part of sternum after a rhombus dissection of the mediastinal fat, pericardium and pleurae, thus avoiding great retrosternal dissection.

4- Using a molder the metallic plate is molded. It will be placed behind the sternum in a way to allow sternal support at an adequate high and to serve as a base for distal extremities of ribs, which will sustain the plate in place.

5- There are sets of metallic plates with one hole on each side through which the steel strands pass to fix the plate on rib extremities. The plates have different measurements from 10 to 25 cm in 1cm intervals as shown below. This 1 cm. difference in length may be fundamental for the sternal support and costal basis.
 
TECHNIQUE
Under general anesthesia a bilateral submammary incision is done (Fig.1), occasionally longitudinal; with a cautery the skin and subcutaneous cell tissue are dissected, going up to the limit of sternal, costal, chondral deformity and down to the inferior limit of chondrocostal deformity, exposing the anterior thoracic and superior abdominal musculatures (Fig. 2 & 3).

The pectoral muscles are released at the medial line by a longitudinal incision and dissected until the chondrocostal articulations in both sides. Downwards the abdominal rectus and the external and internal obliques are disinserted to expose the inferior portion of sternum, ribs and costal cartilages (Fig.4).

With a cautery the perichondrium is incised and a subperichondral resection of cartilages involved in the deformity is performed with the adequate detachments (Fig. 5). Normally resection is done from the third to the seventh costal cartilage. Resection is always bilateral, even in Asymmetric Pectus; cause the side in which the cartilages are preserved the sternum will be pushed forward in the case of Pectus Carinatum and backwards in Pectus Excavatum, promoting deformity recidivism. Since the perichondrium is the germinative element of cartilage, what means it produces cartilage, in about 3 months the costal cartilage will be restored in the new position.

The xiphoid process is released. When is too angled it must be resected. In Classic pectus Carinatum, most of the time the sternum takes the normal position, not being necessary to make an osteotomy. In a pectus Carinatum where the sternum does not take the normal position – Mixed Pectus and Pectus Excavatum – an osteotomy to correct the deformity is needed. Normally, one osteotomy is enough, but there are cases that need 3 and even 4 osteotomies (one of our cases). (Fig.6)

The sternum is released from the loose mediastinal tissue, pericardium and pleurae, bilaterally taking care not to perforate them. The osteotomy/osteotomies are always done on the anterior part with chisel and hammer. The mediastinal structures are protected with valves, varying the inclination of osteotomy/ osteotomies as needed (Fig.7). In most cases we only resect the anterior cortical of sternum, so the posterior cortical will help to maintain the sternum in position. To adequately correct the defect it is necessary, sometimes, to make osteotomy in both corticals. The osteotomy fixation is done with two strands of steel 4.
At this point, bone and chondral extremities are regularized so they will not produce saliences in the skin and thoracic contour will be the most perfect possible. The excess of perichondrium is pleated with polipropilene 0, to stabilize the anterior thoracic wall and help the sternum to stay in the right position (Fig.9).

The pectoral muscles are stitched together at the medial line and the abdominal muscles at the inferior margin of the pectoral musculature (Fig.10). The subcutaneous cellular tissue is brought near through uncolored polipropilene 000 and the intradermal suture is performed. Two Suctor drains are placed, one in the subcutaneous cellular tissue and other at the submuscular level (Fig11).
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Fig. 1
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Fig. 2
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Fig. 3
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Fig. 4
 
TECHNIQUE
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Fig. 5
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Fig. 6
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Fig. 7
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Fig. 8
 
TECHNIQUE
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Fig. 9
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Fig. 10
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Fig. 11
 
PATIENTS SUBMITTED TO STERNOCHONDROPLASTY
Because of the experience with about 200 cases, the Cajurú Hospital Thoracic Surgery Services from the Pontifical Catholic University of Paraná is the leader among the Brazilian Thoracic Surgery Services receiving recognition as a reference center in the surgical treatment of the anterior thoracic wall. This experience is summarized in Table 1.

It should be highlighted that only 10% of the patients that come for orientation are operated because most deformities are small and do not need a surgical treatment.

The patients’ age range was from 4 to 40 years of age, and 77.9% were operated between 10-20 years old (Table 2). There was a prevalence of 6 male patients to 1 female patient.
 
TABLE 1
 
TABLE 2
 
PECTUS CARINATUM EXAMPLES
 
EXAMPLE 1 - SYMMETRIC PECTUS CARINATUM
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Preoperative
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Postoperative - 1 year
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Postoperative - 5 years
 
EXAMPLE 2 - LATERAL RIGHT PECTUS CARINATUM + MAMMARY HIPOPLASIA
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Preoperative
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Postoperative
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Postoperative
 
EXAMPLE 3 - SUPERIOR PECTUS CARINATUM
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Preoperative
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Postoperative - 6 months
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Postoperative - 5 years
 
EXAMPLE 4 - SUPERIOR PECTUS CARINATUM
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Preoperative
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Postoperative
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Postoperative - 5 years
 
EXAMPLE 5 - INFERIOR PECTUS CARINATUM
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Preoperative
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Cardiac Surgery
Postoperative
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Postoperative
Pectus Correction
 
EXAMPLE 6 - INFERIOR PECTUS CARINATUM
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Preoperative
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Preoperative
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Imediat Postoperative
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Imediat Postoperative
 
EXAMPLE 7 - LATERAL PECTUS CARINATUM
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Preoperative
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Imediat Postoperative
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Preoperative
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Imediat Postoperative
 
EXAMPLE 8 - INFERIOR PECTUS CARINATUM
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Preoperative
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Postoperative - 30 days
 
PECTUS CARINATUM
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Preoperative
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Postoperative - 1 month
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Preoperative
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Postoperative
 
RESULTS
Surgery results were classified as follows:
a) bad - when recidivism of deformity happened;
b) fair – when patients and family do not like the result; when a hypertrophic scar occurred ; and when a satisfactory contour of the thoracic wall was not reached;
c) good- when patients, families and surgery team considered they reached the targeted result.

Fifty-nine Pectus Excavatum patients (95.6%) were satisfied with the esthetic result attained. There was one case of recidivism (1.6%) what is considered a bad result. It was a case of Wide Pectus Excavatum, which did not receive a retrosternal metal plate and the patient refused to have another surgery.

Two patients (3.2%) were not completely satisfied with the esthetic result, one of them because of the occurrence of hypertrophic scar (fair result).

One hundred and ten patients (95.6%) carrying Superior Pectus Carinatum and Inferior Pectus Carinatum submitted to surgery were completely satisfied with the attained esthetic results (good result).

Three of them (2.6%) were not completely satisfied with esthetic results obtained (fair result) one of them because of a resulting hypertrophic scar. Two (1.7%) patients showed sternal depression in late postoperative, one had Superior Pectus Carinatum and other Inferior Pectus Carinatum.

A global analysis of the results showed that the result was considered good in 175 patients (95.6%). Five (2.7%) patients showed a fair result. Three (1.6%) presented a bad result, one because of Pectus Excavatum recidivism and two because of sternal depression after Pectus Carinatum surgery.

One patient (0.5%) had a tattoo done on top of the cicatrix even though the surgical and esthetical results were considered good (example).
 
EXAMPLE
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Preoperative
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Postoperative - 1 year
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Postoperative - 5 years
 
COMPLICATIONS
Several complications have been reported: seroma, hematoma, wound infection, pneumothorax, hemoptysis, hemopericardium, cardiac tamponade, skin necrosis, cheloid, sternal necrosis, atelectasis, cardiac chambers lesions, fracture and dislodging of plate or pins, recidivism and Acquired Jeune Syndrome. In our casuistics 14 (7.6%) postoperative complications were found. Two cases of hypertrophic scars were treated by resection and betatherapy after the sixth day on had excellent esthetic results. Eight patients presented seroma on immediate postoperative and needed punches for relieve. Two patients complained of chest pain in postoperative. One female patient presented mid-thoracic pain, at the thoracic spine level due to the catheter used for postoperative epidural analgesy. Another patient presented a partial dehiscence of suture, at the medial portion of the incision, which was solved with a local curative and approximation of the borders with drape.
 
CONCLUSION

Taking in consideration the excellent esthetic results obtained (94.9%), in about 200 patients, the low rate of recidivism in our personal series (0.5%) and a non-occurrence of serious complications and the psychological and postural disturbances that are present in many carriers of Pectus Excavatum and Pectus Carinatum, we think that surgery indication is for patients with accentuated thoracic deformities and if patients really want to do it, except for cases of Symmetric or discreet Lateral Pectus Carinatum in young patients still growing. I would like to point out that only 10% of patients referred to us are operated.

 
MARLOS DE SOUZA COELHO is a national reference in the surgical treatment of deformities on the anterior thoracic wall known as PECTUS EXCAVATUM AND PECTUS CARIANTUM, which he has been studying for 25 years. During this period he has evaluated around 2000 patients.



 
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