A family medicine physician contacted the on-call pediatrician about a 14-year-old male who was worried about his diagnosis of pectus carninatum. He had always had a prominent anterior chest but it had become more pronounced during puberty and he was now very self-conscious. The family medicine physician wanted to know when it was appropriate to refer to a surgeon and if the surgery staff at the regional children’s hospital did surgeries for this problem. The pediatrician said that she knew that there were open- and closed- surgical procedures for the problem and also thought that bracing was an option but she herself hadn’t treated anyone with this problem recently. The pediatrician recommended referring to the surgical services at the hospital. She also suggested considering some psychological support services for possible anxiety and body image problems the patient may be having. The family medicine physician said that he had already suggested the psychological services to the family and they were considering doing it. He was going to send the patient to the surgeons.
Pectus carinatum (PC) is the second most common congenital chest wall deformity after pectus excavatum. It presents usually in early adolescence around puberty, most commonly in males (4:1) and occurs 1 time in every 2500 persons. There are two common variants of the sternum and adjacent costal cartilages protrusion: chondrogladiolar (the body of the sternum protrudes, ie the gladiolus), and chondromanubrial (the superior part of the sternum protrudes). There can also be a mixed variant where the manubrium protrudes and the gladiolus is depressed. Most patients have symmetric deformities but they can be unilateral.
The etiology is unknown but felt to be due to abnormal costal cartilage growth. A positive family history is found in ~25% of patients and PC is associated with Marfan and Noonan syndromes. Patients usually present during the adolescent growth spurt and usually have no other symptoms than the protruding chest. Sometimes patients can complain of chest wall discomfort particularly when lying prone. Heart palpitations or respiratory symptoms are unusual. The more common problems are disturbed body image and inability to wear clothing (particularly bras in females) with resulting decreased quality of life. The evaluation mainly is the history and clinical physical examination. Plain chest radiographs are usually enough to plan surgeries if needed. Patients with other symptoms or with mixed variations may need a cardiac evaluation and/or pulmonary function testing.
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Once puberty has stopped, the PC progression is minimal. Age, pubertal status and chest wall flexibility helps determine the treatment options which are usually:
- Watchful waiting and support – Many patients and families choose this option once they understand the natural history. Pectoral muscle strengthening can also be used help change the chest contour and make the PC appear less noticeable.
- External bracing – This is the treatment option usually recommended for pre-pubertal and pubertal adolescents as it is non-invasive. The side effects are skin irritation and it is effective. There are multiple regimens but generally the bracing needs to be used multiple hours a day and for many months. Bracing is often continued until puberty has ceased.
- Surgery – The Ravitch procedure (osteotomy and costal cartilage resection) and its variations have been used for more than 50 years. The Nuss procedure (stainless steel bar is used to compress the chest wall) is also used. The Nuss procedure has the advantage of being a minimally invasive technique and thus has aesthetic advantages.
Questions for Further Discussion
1. How is pectus excavatum treated?
2. Besides pectus excavatum and pectus carinatum, what other chest wall deformities are there?
3. List different cartilage disorders.
- Symptom/Presentation: Mass or Swelling
- Specialty: Surgery
- Age: Teenager
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
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To view videos related to this topic check YouTube Videos.
Blanco FC, Elliott ST, Sandler AD. Management of congenital chest wall deformities. Semin Plast Surg. 2011 Feb;25(1):107-16.
Obermeyer RJ, Goretsky MJ. Chest wall deformities in pediatric surgery. Surg Clin North Am. 2012 Jun;92(3):669-84, ix.
Desmarais TJ, Keller MS. Pectus carinatum. Curr Opin Pediatr. 2013 Jun;25(3):375-81.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.
26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital