eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Pectus Carinatum

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
Contributor Information and Disclosures

Updated: Aug 25, 2008

Introduction

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.

Clinical

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.

• Search for CME/CE on This Topic »

• Noonan Syndrome (Pediatrics: Genetics and Metabolic Disease)
• Chest Wall Deformities (Pediatrics: Cardiac Disease and Critical Care Medicine)
• Mitral Valve Prolapse (Pediatrics: Cardiac Disease and Critical Care Medicine)
• Mucopolysaccharidosis Type IV (Pediatrics: Genetics and Metabolic Disease)