Pectus Carinatum

Pectus carinatum is a forward protrusion of the sternum (breast bone) and/or ribs, which may give the person a "bird-like" appearance and has thus been called "pigeon breast" in the past.  It is a developmental anomaly (occurring during childhood, during growth and development) and is usually not present at birth. 

Incidence
This condition is relatively rare, affecting approximately 1 in 10,000 persons.  It is roughly 1/10th as common as pectus excavatum, the condition which causes the sternal bone to sink backwards, toward the heart and lungs.  Boys are more frequently affected than girls.  Typical patients develop this problem early in adolescence, between the ages of 10 and 14 years. There may be a family history of other affected relatives.

Considerations
Pectus carinatum is most frequently a solitary abnormality and is usually not associated with other serious genetic disorders or syndromes.  Rare patients, however, may have other connective tissue disorders, such as Marfan's syndrome.  Major genetic work-ups, with multiple tests and consultations, are usually not indicated when evaluating pectus carinatum patients.

Some children with pectus carinatum are asymptomatic.  Others can develop shortness of breath with exertion, chest pain, recurrent respiratory infections, or asthma.  Others may have significant psychological issues, with self-esteem problems and embarrassment concerning the unusual appearance.

What Can Be Done?
Bracing: Many children with flexible, growing chest bones and cartilages, are excellent candidates for nonoperative management of this condition with external compression bracing.  Since 2006, our surgeons have been providing this treatment, in collaboration with local prosthetics professionals.  The brace can be custom-fit to the child and worn beneath the clothing.  In appropriate patients, we believe bracing should be the initial treatment offered to the child.  With this approach, surgery can be avoided  roughly 70% of the time.

Surgery: Some children fail to respond to external bracing.  This most often occurs because the chest tends to "stiffen up" as children go through adolescence and becomes less malleable.  Other children are poor candidates for bracing due to asymmetry, when one side of the chest sticks forward much more than the other side.  Surgical repair of symptomatic pectus carinatum that has failed compression therapy, is usually performed using a modification of the Ravitch approach, originally described in the 1950's.

Unfortunately, many commercially available medical textbooks touch only superficially on pectus carinatum. This is understandable, since this deformity occurs only very rarely, and is perhaps only one tenth as common as pectus excavatum. Medical textbooks typically summarize outdated, poorly planned and designed studies which failed to show significant improvements in pulmonary function tests following surgical repair and thus often conclude that pectus carinatum repair is a "cosmetic procedure" without true physiologic benefit. As we now know, that conclusion is probably flawed. Pectus carinatum may cause chest wall distortion, with dyspnea (shortness of breath), exercise limitation, frequent respiratory infections, and (perhaps most commonly) chest wall pain.

We believe there are several important studies of note on this topic and would be happy to share these with you:

1. The first good retrospective pectus carinatum patient series, by Welch and Vos from The Children's Hospital of Boston, published in the Journal of Pediatric Surgery in 1973, describing their experience with 26 children over a 19 year period. As the authors noted in their introduction, "in some cases (pectus carinatum) results in a rigid chest with increased anterior posterior diameter locked in nearly full inspiration. Respiratory efforts are inefficient, being accomplished only with the diaphragm and accessory muscles of respiration. There may be gradual loss of lung compliance, progressive emphysema, and superimposed pulmonary infection. In addition, the deformity is unsightly, occasionally painful, and psychologically distressing." Preoperative symptoms included respiratory infections or asthma in 38% and chest pain in 19%. Postoperatively, 88% of patients had "permanent and satisfactory correction of the deformity" (12% were lost to follow-up).
2. The series by Wolf et al from the University of Minnesota, published in Minnesota Medicine in 1987, with 191 children. Of the 34 pectus carinaturm patients, 18% had preoperative exercise intolerance and 48% complained of anterior chest wall pain. Postoperatively, pain was relieved in 11 patients (69%) and improved in 4 (25%). Only one patient (6%) with preoperative chest wall pain did not improve.
3. The series by Golladay and Golladay from the University of Michigan, published in the Indian Journal of Pediatrics in 1997, summarizing their 22 year experience with more than 300 children with chest wall deformities. As the authors noted, "pectus carinatum is often associated with symptoms of anterior chest wall discomfort over the greatest sternal protrusion. Some patients report a neuropathic pain related to compression of intercostal nerves between adjacent costochondral cartilages. This pain typically occurs with truncal rotation, is sharp and brief in quality, and radiates to the epigastrium." In their 19 children with pectus carinatum who underwent surgical repair, pain was relieved in 9 of the 13 patients (69%) who had pain preoperatively, and improved in 3 (16%).
4. The largest series of patients reported with only perctus carinatum, by Fonkalsrud and Beanes, from the University of California at Los Angeles, published in the World Journal of Surgery in 2001, detailing their experience with 90 patients over 30 years. All patients were symptomatic; 84 (93%) had exertional dyspnea and exercise limitation, 52 (58%) had frequent respiratory infections, 24 (27%) had asthma, and 38 (42%) had chest discomfort. Postoperatively, all patients experienced alleviation of respiratory symptoms and chest discomfort and diminished exercise limitation.
5. The series by Mansour, et al from Emory University in Atlanta, published in the Annals of Thoracic Surgery in 2003, detailing their experience with 77 adult patients over 30 years. Of the nine patients operated on for pectus carinatum, "all patients exhibited marked clinical improvement, with associated low morbidity. All patients underwent correction for medical reasons."

In summary, recent studies of children and young adults with pectus carinatum, before and after surgical repair, have documented significant improvements in preoperative symptoms, including exercise intolerance, recurrent respiratory infections, and anterior chest wall pain. All studies are from reputable authors and are peer reviewed.

If you have any questions concerning this topic, please feel free to contact us at any time and arrange an evaluation in our office.