Pectus carinatum: surgery or bracing

Mary E Cataletto MD, Chief Editor: Denise Serebrisky MD, Medscape Updated April 20, 2018

Overview

Background

Pectus carinatum (ie, carinatum or keel-shaped deformity of the chest) is a term used to describe a spectrum of protrusion abnormalities of the anterior chest wall.

The deformity may be classified as either chondrogladiolar or chondromanubrial, depending on the site of greatest prominence. Lateral deformities are also possible.

Hippocrates described the carinatum deformity as a “sharply pointed chest” and reported that patients became “affected with difficulty breathing.” Symptomatic patients report dyspnea and decreased endurance. Some develop rigidity of the chest wall with decreased lung compliance, progressive emphysema, and increased frequency of respiratory tract infections.^[1] Many affected patients have no physical complaints; however, concerns about body image have been associated with low self-esteem and a decreased mental quality of life.^[2] Cosmetic concerns can be significant factors in opting for correction.

Barrel chest deformities with increased anteroposterior (AP) chest diameters can be seen in obstructive forms of chronic pulmonary disease, such as cystic fibrosis and untreated or poorly controlled asthma.

Pathophysiology

Until recently, most cases of pectus carinatum deformity were thought to be asymptomatic. However, little is known about the cardiopulmonary function. In 1989, Derveaux reported a series of patients with no significant preoperative or postoperative respiratory compromise.^[3]

However, some patients develop a rigid chest wall, in which the AP diameter is almost fixed in full inspiration. In these patients, respiratory efforts are less efficient. Vital capacity is reduced, and residual air is increased. Alveolar hypoventilation may ensue, with arterial hypoxemia and the development of cor pulmonale. As the lungs lose compliance, incidence of emphysema and frequency of infection are increased.

Most recently, Fonkalsrud (2008) reported his personal experience of 260 patients, all of whom were symptomatic.^[4] Symptoms that were reported included dyspnea, exertional tachypnea, and reduced endurance.In 1990, Iakovlev and colleagues studied the cardiac functions of 70 patients with pectus carinatum deformity.^[5] Mitral valve prolapse was identified in 97%. Rhythm disturbances and decreased myocardial contractility were less frequently observed, along with other cardiac and hemodynamic changes. Cardiac and hemodynamic changes were more commonly observed in patients with chondromanubrial prominence.

Epidemiology

Frequency

United States
Pectus excavatum is more common than the carinatum deformity. The overall prevalence of pectus carinatum is estimated at 0.06%.^[6] Fonkalsrud (2008) reported that at least 25% patients have a positive family history of chest wall deformity.^[4] Pectus carinatum can also be seen in association with Marfan syndrome and congenital heart disease.

International
The percentage of chest wall deformities represented by pectus carinatum are greater in reports from Brazil ^[7] and Argentina.^[8]

Mortality/Morbidity

Psychological and cosmetic concerns are the most prominent reasons for initial consultation. However, Fonkalsrud (2008) reported that surgical repair is rarely performed only for cosmetic reasons.^[4] Morbidity in later years includes cardiac and hemodynamic changes.

Race

The conditions is more frequent in whites and is uncommon in blacks and Asians.

Sex

Males are affected 4 times more frequently than females. Because this deformity may occur either in isolation or as part of a syndrome, identifying a single etiology for the male predominance is difficult.

Age

Although pectus carinatum has been described at birth, it is most frequently identified in mid childhood. The deformity often worsens during the adolescent growth spurt.

Clinical Presentation

History

Parents or the patient may report that pectus carinatum has been present since birth or early childhood, but most children present at age 11-15 years.

The degree of deformity may worsen during adolescence, and most patients are asymptomatic.

Once adult growth has occurred, the severity of the deformity generally remains stable.

Symptomatic patients report exertional dyspnea and tachypnea as well as decreased endurance. In one series, asthmatic symptoms were reported by 22% of patients.^[4]

Musculoskeletal chest pain and tenderness when lying in the prone position can also occur.^[9]

Patients may be affected by low self-esteem, poor body image, and decreased mental quality of life.^[2]

Physical

Two main types of pectus carinatum deformities have been described: chondrogladiolar and chondromanubrial. The most common is the chondrogladiolar form, in which there is a symmetric protrusion of the sternum and costal cartilages.^[9]

Some authors think that a lateral category should also be included. The image below shows an example of a child with a lateral deformity.

In addition to the descriptive findings of anterior chest wall prominence, poor chest wall expansion with inspiration may be observed.

Pectus carinatum. Marlos de Souza Coelho, Paulo de Souza Fonseca Guimarães. 2007;33(4):463-474. Brazilian Journal of Pulmonology

Causes

Etiology has not been established; however, the increased incidence of positive family history and associated anomalies has suggested an abnormality in connective tissue development.^[10]

Pectus carinatum deformities are associated with overgrowth of the rib cage during development of the chest wall.

Differential Diagnosis

Diagnostic Considerations

Diagnosis of pectus carinatum is clinical and is based on descriptive findings identified during the physical inspection of the chest. It may occur as an isolated anomaly, in association with congenital heart disease, or with another skeletal anomaly (20% scoliosis). Mixed deformities can be observed in Poland syndrome. Approximately 25% of patients have a positive family history of chest wall deformity. Less frequently, pectus carinatum has been associated with Morquio syndrome, hyperlordosis, and kyphosis.

Workup

Imaging Studies

Radiographic imaging should include 2 view chest radiographs: posteroanterior and lateral images. A chest radiograph of a patient with pectus carinatum is shown in the image below.

Additional imaging with either a chest CT scan or MRI may also be helpful. CT scanning of the chest in an individual with pectus carinatum reveals an increased anterioposterior chest wall diameter.^[11]

The Haller method may be used to determine severity index, as follows: width of the chest divided by distance between the sternum and spine at the same level; this may help to predict those individuals who will benefit from surgical intervention.

Other Tests

In patients with pectus carinatum, pulmonary function studies may be tailored to address concerns about clinical symptoms and the appearance of the chest wall upon examination. Data on pulmonary and exercise physiology in patients with pectus carinatum deformities are limited. However, children with barrel chests usually have obstructive ventilatory defects. This underscores the importance of preforming complete pulmonary function testing, including prebronchodilator and postbronchodilator spirometry, lung volumes, and diffusion capacity. Exercise testing may complement these studies.

In 1982, Castile described one patient who reported exercise intolerance in his series of symptomatic pectus deformities.^[12] His pulmonary function studies revealed flow rates and lung volumes within the reference range. Derveaux’s 1989 series also reported a patient with no significant respiratory compromise at the time of his study.^[3]

Progressive exercise studies may also be helpful in evaluating the exercise-related symptoms and exertional tolerance.

Electrocardiography and echocardiography may be considered if congenital heart disease is suspected. Iakovlev’s study reported 70 patients with pectus carinatum deformity. Of these, 97% had echocardiographically documented mitral valve prolapse.^[5] Hemodynamic and cardiodynamic changes were also observed in some patients, as well as decreased myocardial contractility. These abnormalities were more frequently observed in the patients with pigeon breast.

Scoliosis series may be considered if clinical features are suggestive of this diagnosis.

Chromosomal analysis and metabolic testing may also be considered if other dysmorphic signs are identified.

Treatment

Medical Care

Most motivated patients with pectus carinatum, especially those younger than 18 years with malleable chest walls, benefit from orthotic bracing, and this is generally the first line of therapy.^[13,14] Success rates of 65-80 % and long-term outcomes with orthotic bracing alone are encouraging.^[9]

A study by Lee at el (2012) describes the preliminary results of 98 children treated using the Calgary Protocol, which involves a self-adjustable, low-profile bracing system used in 2 phases. The first phase involves 24 h/d bracing until correction is achieved. The second phase is a maintenance phase during which the brace is worn only at night until axial growth is complete.

Twenty-three children completed treatment with good patient satisfaction and improved appearance, suggesting that when used in this fashion, it is an effective treatment for pectus carinatum. Therapy failed in 42 children, owing to either noncompliance or they were lost to follow-up. Two required surgical intervention after bracing failed to correct the problem. Additional studies and follow-up of the children still on protocol is important.^[15] A study by Wahba et al reported that less intensive bracing.^[16]

For older patients with more rigid chest walls, bracing may not be effective and surgery may be the initial consideration.

Casting followed by bracing or bracing alone eliminates the risks of surgery and anesthesia and does not preclude surgery if unsuccessful.

Surgical Care

Endoscopic resection of costal cartilage with a sternal osteotomy

Because many corrections are performed for cosmetic reasons, decreasing the size of incisions is important.

In 1997, Kobayashi reported 2 patients in whom the pectus carinatum deformity was corrected with limited incisions using an endoscopic approach.^[15] They suggest that this approach is better indicated in preschool-aged children because of their skin quality and tone, as well because of the increased ease of costal dissection compared with adult patients.

In 2008, Fonkalsrud reported a series of 260 patients who underwent surgical correction of pectus carinatum deformities over a period of 37 years.^[4] He concluded that, over time, the trend towards less extensive open techniques has resulted in “low morbidity, mild pain, short hospital stay and very good physiologic and cosmetic results.” His study included both pediatric and adult patients.

Open surgical repair

Various methods have been described. The reader is referred to Fonkalsrud (2008),^[4] de Matos (1997),^[17] Shamberger (1987),^[18] Del Frari (2011),^[19] and Cohee (2013) ^[20] for further details.

Consultations

Pectus carinatum has been associated with congenital heart disease. In these patients, and in those with suspected or identified cardiac pathology, preoperative cardiology evaluation is recommended.

Exercise testing may be performed in consultation with either a cardiologist or a pulmonologist.

Symptomatic patients with exertional dyspnea, tachypnea, or decreased endurance, as well as those with asthma symptoms, benefit from a pulmonology evaluation.

Individuals with pectus carinatum who have significant concerns about their body image or low self-esteem can benefit from psychological counseling.

Activity

Symptomatic patients may report decreased exercise tolerance and exertional dyspnea, which may limit activity. Fonkalsrud’s series (2008) reported improvement in exertional symptoms and endurance in all symptomatic patients within 3-6 months of surgical repair.^[4]

Fonkalsrud’s recommendations for postoperative activity include the following: ^[4]

• Use incentive spirometer and encourage periodic deep breaths.
• Limit twisting movements of the chest for at least 4 months postoperatively.
• Avoid rapid elevation of the arms overhead for at least 4 months postoperatively.
• Encourage lower extremity exercise (may begin within first 2 wk after surgery).
• Light weights may be used to strengthen biceps and deltoids; the use of chest and abdominal muscles may be increased later (after 3-4 wk).
• Gym classes are not indicated for 5 months after surgery in school-aged children.
• Long-term recommendations include stretching exercises that involve pulling the shoulder

Medication Summary

Drug therapy currently is not a component of the standard of care in pectus carinatum. (See Treatment).

Follow-up

Further Outpatient Care

Long-term activity recommendations include stretching.

Further Inpatient Care

For information regarding these indications in pectus carinatum, (see Activity).

Complications

Complications vary according to treatment selection.

Ill-fitting braces can be associated with skin irritation and skin breakdown.

Shamberger reported a 3.9% complication rate with open surgical repair.^[18] Complications include pneumothorax (2.6%), wound infection (0.7%), atelectasis (0.7%), and local tissue necrosis (0.7%). The mean postoperative stay was 5.8 days.

Fonkalsrud (2008) reported shorter hospital stays (mean, 2.6 d), mild postoperative pain, and low complication rate with limited resection and immediate chest stabilization.^[4]

Prognosis

In prepubertal children with pectus carinatum who are compliant with bracing, success rates are excellent (up to 80%).

Excellent results (97.4%) have been reported by Fonkalsrud (2008) in patients who underwent surgical correction using a very limited resection of deformed cartilage and immediate chest stabilization.^[4] In addition, he reported less postoperative pain, shorter hospital stays, lower complication rate, and decreased cost. Furthermore, he reported satisfactory cosmetic results with the less extensive repair, as well as a high rate of improvement in exertional symptoms compared with more extensive open surgical procedures.

Recurrences are rare.

Responses to quality-of-life questionnaires in patients who had undergone minimally invasive repair of their pectus deformity supported a positive impact on psychosocial function.^[21]

Patient Education

Exertional symptoms may develop with pectus deformities and may not always be identified with standard pulmonary function testing.

References

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