Pectus Excavatum

Pectus excavatum describes an abnormal configuration of the chest, where the sternum (breast bone) pushes into the heart and lungs, giving the chest a "caved-in" or sunken appearance.  It is a developmental anomaly (occurs during development of the skeleton, during childhood) and is usually not present at birth.  It has also been called "funnel chest."

Considerations
Pectus excavatum is relatively rare, affecting roughly 1 in 1,000 children.  Boys are more frequently affected than girls.  There may be a family history of the disorder, with other affected relatives.

Although there is no proven cause for this disorder, it is felt that the cartilages, which connect the ribs to the sternum, grow "too long" during childhood and adolescence and, as a result, are too large for the child's chest cage, and that these abnormal cartilages subsequently "push" the sternal bone inward.

Most children with pectus excavatum have this condition as an isolated defect.  Rare children have other associated conditions, such as Marfan's syndrome, Poland's syndrome, or various connective tissue disorders.  Other children can develop pectus excavatum as a consequence of complicated surgery during early childhood, such as repair of a congenital diaphragmatic hernia.

Symptoms
Some children with pectus excavatum are completely asymptomatic.  Others develop symptoms which are most likely due to compression of the heart and/or lungs, such as shortness of breath with exertion, chest pain, palpitations (rapid heart rate), recurrent respiratory infections, or asthma.  Other children may develop significant psychological distress, with self-image problems and embarrassment.

Unfortunately, many commercially available medical textbooks touch only superficially on pectus excavatum. They typically summarize outdated, poorly-planned and designed studies which failed to show significant improvements in pulmonary function tests and thus often conclude that pectus excavatum repair is a "cosmetic procedure" without true physiologic benefit. As we now know, that conclusion is probably flawed.


The primary physiological problem with pectus excavatum may not be compression of the lungs but, rather, may be compression of the right ventricle of the heart, which results in decreased cardiac output and impaired blood flow to the lungs during vigorous exercise. Correction of pectus excavatum has been clearly shown to improve this.

We believe that articles of note include:

  1. The first good study, by Beiser et al from the National Heart and Lung Institute, published in the New England Journal of Medicine in 1972, with volunteers undergoing invasive testing (cardiac catheterization) before and after pectus repair, demonstrating an average 38% improvement in cardiac output during intense upright exercise. The authors concluded that “pectus excavatum can reduce the pumping capacity of the heart during upright exercise, and hemodynamic improvement occurs after surgical correction.”
  2. The elegant study, by Kowalewski et al from the Military Medical Academy of Lodz, Poland, published in the Journal of Thoracic and Cardiovascular Surgery in 1999, using echocardiography, documents statistically significant changes in right ventricular volume indices after surgery with increases in right ventricular systolic, diastolic, and stroke volumes.
  3. The study, by Haller and Loughlin from Johns Hopkins University, published in the Journal of Cardiovascular Surgery in 2000, following their personal experience with surgical correction of over 700 patients over a 40-year period, showing that 66% of pectus patients with preoperative complaints of exercise limitations were significantly improved after surgery. More importantly, after correction, pectus patients were able to exercise longer and had higher oxygen pulse than before surgery. The authors conclude that the “results suggest that pectus excavatum repair improves cardiopulmonary function during vigorous exercise.”
  4. The study, by Fonkalsrud et al from UCLA, published in Annals of Surgery in 2000, following their personal experience with 375 patients over a 35-year period, showing that, with a mean follow-up of 12.6 years, all patients with preoperative respiratory symptoms, exercise limitation, and chest pain experienced improvement. Vital capacity increased a mean of 11% within 9 months in 35 patients evaluated. Seventy-four of 90 preoperative functional heart murmurs were no longer audible after surgery.
  5. The first report from the Nuss group, from the Hospital of the King’s Daughters in Norfolk, Virginia, published in the Journal of Pediatric Surgery in 1998, describing the new Minimally Invasive Technique for Correction of Pectus Excavatum, detailing the experience with the initial 42 patients. Of the 30 patients who had progressed at that time to bar removal (completion of the surgical treatment), 87% had excellent or good results.
  6. The study from Coln et al, from the University of Texas Medical School in Dallas, published in the Journal of Pediatric Surgery in 2006, involving 123 patients over 6 years. 95% of the patients had documented cardiac compression on preoperative echocardiograms. Postoperatively, all symptomatic patients improved. More importantly, echocardiograms documented resolution of cardiac compression in 93% of the patients studied.
  7. The elegant study of 120 patients from Neviere, et al, from France, published in the European Journal of Cardio-thoracic Surgery in 2011, documenting dramatic impairments in cardiac function caused by pectus excavatum, with significant improvements in maximal oxygen uptake after surgical repair.
  8. The study of 75 teenagers done by Tang, et al, from Denmark, published in the European Journal of Cardio-thoracic Surgery in 2012, showing heart compression (with a lower cardiac index) in pectus patients, with significant improvements one year following surgery.
  9. The study from Tardy, et al, from France, published in the Journal of the American College of Cardiology in 2015, showing that pectus excavatum patients matched by age, weight, and height to controls, have (on average) 25% less ability to perform vigorous upright exercise due to decreased stroke volume (blood output from the heart) due to cardiac compression.
  10. Finally, the Meta-analysis article from Malek et al (a group of “non-surgeon” physiologists, kinesiologists, and psychologists), published in Chest in August 2006. These authors reviewed the entire world’s published literature on this topic and concluded that surgical repair clearly improves cardiovascular function in a statistically significant fashion in these children and that this definitely “contradicts arguments that surgical repair is primarily cosmetic…”

In summary, recent studies of children and young adults with pectus excavatum, before and after surgical repair, have documented significant improvements in heart function, exercise tolerance, and quality of life. All studies are from reputable authors and are peer-reviewed. The Ravitch technique of pectus excavatum repair, as popularized recently by Haller (Johns Hopkins) and Fonkalsrud (UCLA) has an extensive history and well-documented success. The Nuss technique, as detailed by Dr. Nuss and performed extensively throughout the United States and Europe, has also been shown to result in similar improvements.


Surgery

Unfortunately, there is no proven non-operative technique for the correction of pectus excavatum. 

The minimally-invasive, or "Nuss" technique, for the repair of pectus excavatum, was originally reported in 1997 and has been performed by surgeons in our department since 1999.  This involves temporary placement of one or more metal bars in the chest to push the sternal bone forward.  These bars are guided between the ribs, into the chest, with use of a thoracoscope (small diameter telescope), inserted into the chest during the surgical procedure through small incisions.  Bars are typically removed after 2 to 2.5 years.

The Ravitch technique of pectus excavatum repair was originally described in the 1950's, was popularized more recently by Haller (Johns Hopkins) and Fonkalsrud (UCLA), and  has an extensive history and well-documented success.  This technique may be preferred in some patients, such as some older children and young adults, and in some patients with very asymmetric deformities. 

Both techniques are performed in the operating room, under general anesthesia, and typically require postoperative recovery stays in the hospital for four to seven days.

 

 

 

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