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Pediatrics: General Medicine > Pulmonology
Pectus Carinatum
Article Last Updated: Aug 25, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
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; 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 pigeon chest, chondromanubrial prominence, carinatum deformity, emphysema, respiratory tract infection, asthma, cystic fibrosis, mitral valve prolapse, Marfan syndrome, congenital heart disease
Background
Pectus carinatum (ie, carinatum deformity of the chest) 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." 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. For some, the major concern is cosmetic. Barrel chest deformities with increased anteroposterior (AP) chest diameters are possible 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.1 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.2 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.3 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%.4 Fonkalsrud (2008) reported that at least 25% patients have a positive family history of chest wall deformity.2 Pectus carinatum can also be seen in association with Marfan syndrome and congenital heart disease.
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.2 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.
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.2
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. Media file 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.
Other Problems to be Considered
Diagnosis of pectus carinatum is clinical and is based on descriptive findings identified during the 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.
Imaging Studies
In pectus carinatum, CT scanning of the chest reveals an increased anterior-posterior chest wall diameter. 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.
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.5 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.1
- 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.3 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
In treating pectus carinatum, both dynamic chest compressors and body casting have been described in limited series and a case report.
- A retrospective study by Frey et al reported success with orthotic bracing in a group of 29 children with chondrogladiolar pectus carinatum, using bracing 14-16 h/d until completion of linear growth or a minimum of 2 years.6 Compliance rate with bracing in this group of children was 90%. Frey et al recommend nonoperative management and bracing as first-line treatment for children with this type of pectus carinatum.
- 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 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.7 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.2 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),2 de Matos (1997),8 or Shamberger (1987)9 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.
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.2
Fonkalsrud's recommendations for postoperative activity include the following:2
- 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 blades posteriorly to improve posture.
Drug therapy currently is not a component of the standard of care in pectus carinatum. See Treatment.
Further Inpatient Care
- For information regarding these indications in pectus carinatum, see Activity.
Further Outpatient Care
- Long-term activity recommendations include stretching.
Complications
- Complications vary according to treatment selection.
- Shamberger reported a 3.9% complication rate with open surgical repair.9 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.2
Prognosis
- Frey et al reported success with nonoperative management of pectus carinatum (chondrogladiolar type) in a small number of patients.6
- 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.2 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.
Patient Education
- Exertional symptoms may develop with pectus deformities and may not always be identified with standard pulmonary function testing.
Medical/Legal Pitfalls
- Failure to diagnose pectus carinatum
- Failure to recognize the association of dyspnea and poor endurance with pectus carinatum deformity
- Failure to recognize that surgical options may be beneficial even after skeletal maturity has occurred
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: Radiograph
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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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- 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. Surgical correction of pectus carinatum: lessons learned from 260 patients. J Pediatr Surg. Jul 2008;43(7):1235-43. [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].
- Mielke CH, Winter RB. Pectus carinatum successfully treated with bracing. A case report. Int Orthop. Dec 1993;17(6):350-2. [Medline].
- Castile RG, Staats BA, Westbrook PR. Symptomatic pectus deformities of the chest. Am Rev Respir Dis. Sep 1982;126(3):564-8. [Medline].
- Frey AS, Garcia VF, Brown RL, et al. Nonoperative management of pectus carinatum. J Pediatr Surg. Jan 2006;41(1):40-5; discussion 40-5. [Medline].
- Kobayashi S, Yoza S, Komuro Y, et al. Correction of pectus excavatum and pectus carinatum assisted by the endoscope. Plast Reconstr Surg. Apr 1997;99(4):1037-45. [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].
- Shamberger RC, Welch KJ. Surgical correction of pectus carinatum. J Pediatr Surg. Jan 1987;22(1):48-53. [Medline].
- Cano I, Anton-Pacheco JL, Garcia A, Rothenberg S. Video-assisted thoracoscopic lobectomy in infants. Eur J Cardiothorac Surg. Jun 2006;29(6):997-1000. [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].
- 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].
- 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.
Pectus Carinatum excerpt Article Last Updated: Aug 25, 2008
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