eMedicine Specialties > Pediatrics: General Medicine > Pulmonology
Pectus Excavatum
Updated: Sep 21, 2009
Introduction
Background
Pectus excavatum, also known as sunken or funnel chest, is a congenital chest wall deformity in which several ribs and the sternum grow abnormally, producing a concave, or caved-in, appearance in the anterior chest wall. Media file 1 illustrates the typical appearance of this deformity in a 16-year-old boy.
A 16-year-old boy with severe pectus excavatum. Note the appearance of the caved-in sternum and lower ribs.
Pectus excavatum is the most common type of congenital chest wall abnormality (90%), followed by pectus carinatum (5-7%), cleft sternum, pentalogy of Cantrell, asphyxiating thoracic dystrophy, and spondylothoracic dysplasia. Pectus excavatum occurs in an estimated 1 in 300-400 births, with male predominance (male-to-female ratio of 3:1). The condition is typically noticed at birth, and more than 90% of cases are diagnosed within the first year of life. Worsening of the chest’s appearance and the onset of symptoms are usually reported during rapid bone growth in the early teenage years. Many patients are not brought to the attention of a pediatric surgeon until the patient and the family notice such changes. The appearance of the chest can be very disturbing to young teenagers. Problems with self-esteem and body image perception are frequently reported in teenaged patients. Psychologic disturbances are not unusual in older patients.
A 10-year-old girl with severe pectus excavatum. In girls, the deformity is of particular concern because of the medial displacement of the breast, resulting in significant asymmetry of the breasts and nipples (cross-eyed appearance of the nipples).
A 10-year-old girl with severe pectus excavatum (see Media file 2). Note the significant asymmetry of the breasts and nipples (cross-eyed appearance of the nipples).
A 12-year-old girl with severe pectus excavatum. Note the significant asymmetry of the breasts. Preoperative photograph.
Pathophysiology
In pectus excavatum, the growth of bone and cartilage in the anterior chest wall is abnormal, typically affecting 4-5 ribs on each side of the sternum. The appearance of the defect widely varies, from mild to very severe cases, and some patients present with significant asymmetry between the right and left sides. The exact mechanism involved in this abnormal bone and cartilage overgrowth is not known, and, to date, no known genetic defect is directly responsible for the development of pectus excavatum. Despite the lack of an identifiable genetic marker, the familial occurrence of pectus deformity is reported in 35% of cases. Moreover, the condition is associated with Marfan syndrome and Poland syndrome.
Frequency
United States
Pectus excavatum occurs in an estimated 1 in 300-400 births, with male predominance (male-to-female ratio of 3:1). Pectus excavatum comprises approximately 90% of all chest wall deformities.
International
Although limited data are available, international frequency is probably the same as that reported in the United States. However, in certain countries (eg, Argentina), pectus carinatum is more common than pectus excavatum.
Mortality/Morbidity
Most patients with pectus excavatum are asymptomatic from a functional standpoint. The degree of cardiopulmonary impairment caused by lung compression and the level of cardiac displacement that results from the caved-in chest are subjects of controversy. Exercise tolerance is frequently reported as normal, but a restrictive pattern in pulmonary function test results can be identified. Cardiac function is usually normal, but mitral valve prolapse has been reported in 20-60% of cases. Echocardiography reveals an improved cardiac index upon exertion after operative repair of the deformity. The long-term health risks of patients who are managed without surgery are not known.Race
Pectus excavatum appears to be most prevalent in whites. Unfortunately, no specific data are available regarding racial distribution; however, clinical observation indicates that treating pectus excavatum in African Americans is unusual.
Sex
The male-to-female ratio is 3:1. Despite such observation, no known genetic factor linked to the X or Y chromosome has been reported.
Age
Most cases of severe pectus excavatum are noticed at birth, with progressive worsening of the child's growth and development. More than 80% of all cases are identified within the first 1-2 years of life. The condition typically becomes much more pronounced at puberty, during the time of rapid bone and cartilage growth. Most patients are brought to medical attention during their teenage years because of the significant change in the appearance of their chest.
Clinical
History
Some patients with pectus excavatum experience chest and back pain that is usually musculoskeletal in origin. The exact cause of the pain is poorly understood. Pectus excavatum and pectus carinatum are frequently associated with scoliosis. Although such association is probably coincidental, the poor posture noted in many patients with pectus deformities may be a key factor in the development of pain.
- Pulmonary function
- Many physicians attribute the symptomatic impairment in pectus excavatum to a decrease in intrathoracic volume secondary to the sunken chest. However, this relationship is difficult to prove because of the wide range of pulmonary function among healthy individuals and the correlation of pulmonary function with physical training and body habitus. There has been scientific evidence reported that demonstrates shortness of breath upon exertion in patients with pectus excavatum, primarily due to the decrease in pulmonary reserve.
- Clinicians have observed that many patients with pectus excavatum tend to become symptomatic during their teenage years or early in adult life. Patients younger than 10 years who have pectus excavatum do not typically experience symptoms associated with shortness of breath.
- Studies of pulmonary function in patients with pectus excavatum have provided the following information:
- A prospective study of preoperative and postoperative pulmonary function following corrective surgery for pectus excavatum is currently underway.1 In 1984, Cahill et al reported that, after operative repair, lung capacity improved little, and maximal voluntary ventilation significantly improved in patients with pectus excavatum who had low-to-normal vital capacities prior to surgery.2 Exercise tolerance was also improved, as measured by total exercise time and maximal oxygen uptake. The heart rate at a given level of work or exercise consistently decreased postoperatively, but oxygen consumption to support an improved efficiency of work was not changed. The observed decrease in heart rate at each workload capacity was a result of increased cardiac stroke volume.
- In 1967, Weg et al evaluated 25 US Air Force recruits with pectus excavatum and compared them with healthy trainees.3 Although the lung compartments and mean vital capacities of both groups were equal, the maximum voluntary ventilation significantly deviated from predicted reference range values (P = 0.005).
- In 1996, Quigley et al studied 36 adolescents (mean age 16 y) with pectus excavatum.4 Quigley et al reported a significantly lower forced vital capacity in the study group than in an age-matched control group. Moreover, an inverse relationship was observed between the vital capacity and the degree of sternal compression, suggesting that such patients would benefit from operative correction of the pectus deformity.
- Cardiac function
- Posterior displacement of the sternum in pectus excavatum can produce a heart deformity, with anterior indentation of the right ventricle. Early pathologic studies demonstrated this finding, and a series of early case reports included cardiac evaluations for patients with severe symptoms. Angiographic studies have demonstrated the sternal imprint on the anterior wall of the right ventricle.
- Several studies have demonstrated limitation of cardiac stroke volume in patients with pectus excavatum, particularly in the sitting, or upright, position. When patients with pectus remain in the supine position (lying flat), no significant impairment to cardiac function is apparent. Further evidence has suggested that operative repair of pectus results in normalization of the cardiac function.
- One study assessed cardiac workload in 13 patients with pectus excavatum.5 The patients were assessed in an upright position on a bicycle ergometer. Findings suggested that, following surgery, most patients could more easily reach the target heart rate during exercise without becoming symptomatic. Many use this observation as substantial evidence that operative repair for pectus excavatum results in improved cardiac function. However, the role of conditioning and subjective response to surgery is difficult to assess.
Physical
The hallmark of pectus excavatum is the caved-in appearance of the anterior chest. As mentioned above, the severity of the defect and the asymmetry of the chest widely vary. Patients may present with a very mild form of pectus excavatum or their sternum may be almost touching the spine. Typically, the lower third of the sternum is more involved, and the upper third may appear fairly normal. A compensatory anterior flaring of the lower ribs at each costal margin is also common. Many patients have associated scoliosis, but this is not directly related to the presence of pectus excavatum.
- Auscultation of the chest: Heart sounds are typically displaced to the left side because of displacement and rotation of the heart. A click sound of mitral valve prolapse may be present. Lung sounds are clear, but the lung sounds may appear diminished at both bases because of decreased pulmonary volumes.
- Pectus posture: The term pectus posture refers to the position assumed by most patients with significant pectus excavatum. They appear to create an anterior curvature of the thoracic spine with the shoulders slumped forward. Whether this is a subconscious maneuver to hide the chest wall deformity or a postural defect directly related to pectus excavatum is unclear. Such positioning of the spine appears to accentuate the pectus excavatum and can generate spine problems related to poor posture and inadequate spinal support. Correcting this posture is quite difficult, even after successful repair of the pectus excavatum.
Causes
The cause of pectus excavatum is unknown. It probably originates from a genetic defect that results in abnormal musculoskeletal growth. The cartilaginous portion of the rib is very likely the main source of this abnormal growth pattern. Abnormalities of rib morphogenesis and growth are the most likely causes of pectus excavatum and pectus carinatum. In pectus excavatum, the sternum is thought to be pushed in by abnormal growth at the articulation with the ribs and cartilage. Again, the exact mechanism that results in this abnormal growth pattern is not known. Increased work of breathing, as is observed in young patients during exercise or play activity, may contribute to the progression of the pectus deformity, particularly during early the teenage years. However, no scientific evidence supports such a theory.
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| Treatment & Medication: Pectus Excavatum |
| Follow-up: Pectus Excavatum |
| Multimedia: Pectus Excavatum |
| References |
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References
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Further Reading
Keywords
pectus excavatum, congenital chest wall deformity, sunken chest, pectus, Marfan syndrome, Poland syndrome, minimally invasive repair of pectus excavatum, MIRPE, Nuss technique, open Ravitch technique for repair of pectus excavatum, mitral valve prolapse, scoliosis, carinatum, pectus posture, bone and cartilage overgrowth, sternal turn-over operation, pectus carinatum, pectus deformity, back pain
