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Pediatrics: General Medicine > Pulmonology
Pectus Carinatum
Article Last Updated: Dec 27, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Associate Professor, Department of Clinical Pediatrics, State University of New York at Stony Brook
Mary E Cataletto is a member of the following medical societies: American Academy of Pediatrics, American Heart Association, and American Thoracic Society
Editors: Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center; Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine; Michael R Bye, MD, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
Author and Editor Disclosure
Synonyms and related keywords:
pectus carinatum, bird chest, chicken breast, chondrogladiolar prominence, Pouter's pigeon chest, chondromanubrial prominence, carinatum deformity
Background
Carinatum deformity of the chest (pectus carinatum) represents a spectrum of protrusion abnormalities of the anterior chest wall. The deformity may be classified as either "chicken breast" (chondrogladiolar) or "Pouter pigeon breast" (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." Since his description, various findings have been reported; the most prominent are cosmetic and psychological. Some patients develop rigidity of the chest wall with decreased lung compliance, progressive emphysema, and increased frequency of respiratory tract infections.
Barrel chest deformities with increased anteroposterior (AP) chest diameters are possible with obstructive forms of chronic pulmonary disease, such as cystic fibrosis and untreated or poorly controlled asthma.
Pathophysiology
Most patients with pectus carinatum deformity are asymptomatic. Little is known about their cardiopulmonary function. In 1989, Derveaux reported a series of patients with no significant preoperative or postoperative respiratory compromise. 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.
In 1990, Iakovlev and colleagues studied the cardiac functions of 70 patients with pectus carinatum deformity. 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.
Frequency
United States
Pectus excavatum is more common than the carinatum deformity. The overall prevalence of pectus carinatum is estimated at 0.06% (Mielke, 1993). Fonkalsrud et al (2002) report that approximately one third of patients have a positive family history of pectus deformities.
Mortality/Morbidity
- Psychological and cosmetic concerns are the most prominent reasons for initial consultation.
- Morbidity in later years includes cardiac and hemodynamic changes.
Race
No racial predilection has been reported.
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.
History
- Parents or the patient may report that the carinatum deformity has been present since birth or early childhood, but most children present at ages 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.
Physical
- Two main types of pectus carinatum deformities have been described: chondrogladiolar and chondromanubrial.
- Some authors think that a lateral category should also be included. Image 1 shows an example of a child with a lateral deformity.
- In most instances, the pectus carinatum is a symmetric deformity. Less often (<35%), asymmetric or mixed deformities may be identified.
- In addition to the descriptive findings of anterior chest wall prominence, poor chest wall expansion with inspiration may be observed.
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.
- A number of other theories have been proposed, including abnormal diaphragmatic development and hypertrophic growth of costal cartilages, ribs, or both.
Neurofibromatosis
Other Problems to be Considered
Diagnosis is clinical and based on descriptive findings identified upon physical inspection of the chest. Remember that this deformity 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.
Other Tests
- 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. His pulmonary function studies showed 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.
- Progressive exercise studies may also be helpful in evaluating the exercise-related symptoms and exertional tolerance.
- Electrocardiography and echocardiography
- These tests may be considered if congenital heart disease is suspected.
- Yakovlev's study reported 70 patients with pectus carinatum deformity. Of these, 97% had echocardiographically documented mitral valve prolapse.
- 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.
Medical Care
- Orthotics
- Both dynamic chest compressors and body casting have been described in limited series and a case report.
- The ideal candidate is a motivated, skeletally immature child with a mild deformity.
- 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 most corrections are performed for cosmesis, 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. They suggest that this approach is better indicated in preschool children because of their skin quality and tone, as well as the increased ease of costal dissection compared with adult patients.
- Open surgical repair
- Various methods have been described.
- The reader is referred to de Matos (de Matos, 1997) or Shamberger (Shamberger, 1987) for further details.
Drug therapy currently is not a component of the standard of care for this condition. See Treatment.
Complications
- Complications vary according to treatment selection. Shamberger reports a 3.9% complication rate with open surgical repair. 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.
Prognosis
- Surgical correction is generally successful and recurrences are few. In Shamberger's 1987 series, the recurrence rate was less than 2%. Early age at time of correction and incomplete correction have both been associated with increased frequency of reoperation.
- Some authors consider orthotics a viable option. If considered, it should be instituted while the chest wall is still compliant. The long-term prognosis of orthotics as a treatment option for pectus carinatum is still unknown.
- More recently, Fonkalsrud et al (2002) reported their experience with adult patients undergoing surgical repair for pectus deformities. They used symptom severity, rather than cosmesis, as their indication for surgery and reported surgical repair as a low-morbidity option with good success reported in postoperative symptom reduction.
Patient Education
- Exertional symptoms may develop with pectus deformities and may not always be identified with standard pulmonary function testing.
Special Concerns
- In adults with functional impairment and severe pectus deformities who did not undergo surgical intervention as children, repair during adulthood is currently an acceptable treatment option.
| Media file 1:
Pectus carinatum. Photograph courtesy of K. Kenigsberg, MD. |
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Media type: Photo
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| Media file 2:
Chest radiograph of a patient with pectus carinatum. Radiograph courtesy of A. Fruauff, MD. |
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Media type: X-RAY
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| Media file 3:
CT scan of a patient with pectus carinatum. CT courtesy of A. Fruauff, MD. |
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Media type: CT
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- Castile RG, Staats BA, Westbrook PR. Symptomatic pectus deformities of the chest. Am Rev Respir Dis. Sep 1982;126(3):564-8. [Medline].
- de Matos AC, Bernardo JE, Fernandes LE, Antunes MJ. Surgery of chest wall deformities. Eur J Cardiothorac Surg. Sep 1997;12(3):345-50. [Medline].
- Derveaux L, Clarysse I, Ivanoff I, Demedts M. Preoperative and postoperative abnormalities in chest x-ray indices and in lung function in pectus deformities. Chest. Apr 1989;95(4):850-6. [Medline].
- Fonkalsrud EW, DeUgarte D, Choi E. Repair of pectus excavatum and carinatum deformities in 116 adults. Ann Surg. Sep 2002;236(3):304-12; discussion 312-4. [Medline].
- Iakovlev VM, Nechaeva GI, Viktorova IA. Clinical function of the myocardium and cardio- and hemodynamics in patients with pectus carinatum deformity [in Russian]. Ter Arkh. 1990;62(4):69-72. [Medline].
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- Lacquet LK, Morshuis WJ, Folgering HT. Long-term results after correction of anterior chest wall deformities. J Cardiovasc Surg (Torino). Oct 1998;39(5):683-8. [Medline].
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- O'Neill JA, Fonkalsrud EW, Coran AG, et al. Pediatric Surgery. New York, NY:. Elsevier Health Sciences;1998.
- Sabiston D, ed. Textbook of Surgery. Philadelphia, Pa:. WB Saunders Co;1997.
- Shamberger RC, Welch KJ. Surgical correction of pectus carinatum. J Pediatr Surg. Jan 1987;22(1):48-53. [Medline].
Pectus Carinatum excerpt Article Last Updated: Dec 27, 2006
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