Pectus excavatum is the medical label for the condition sometimes called "funnel chest", a depressed or sunken breastbone (sternum). It is one of the most common human bone deformities -- doctors suggest around 1 of every 400 people have some degree of PE. The deformation ranges from a very mild, centered "dent" in mid-chest, with few symptoms -- to an extremely deep depression forcing the heart over to one side and impairing breathing and heart function and making any exertion painful. Pectus carinatum is a related condition in which the breastbone and ribs protrude more than normal. As with PE, there's a wide range of degrees of severity with PC.
The cause of pectus is not known. What is known is that at least the tendency to develop PE or PC seems to "run in families". Many people with pectus discover that a parent, often their father, had a mild case. It is also a feature in congenital conditions like Marfan's Syndrome and Ehlers-Danlos Syndrome. Pectus may also occur as "isolated" -- that is, not part of a system-wide syndrome but something that's happened on its own. Both PE and PC reflect an underlying problem with connective tissue formation.
Moderate-to-severe PE is often apparent at birth -- but it also may be so mild that it's not really noticed until the body starts growing rapidly at puberty. It commonly worsens through childhood and into the teens, with the deformation stabilising around age 18. After that no further deformation of the bones occurs, although related heart and respiratory problems may continue to worsen with age.
Pectus can have a profound detrimental effect on quality of life. With moderate and certainly with severe PE, the depressed breastbone interferes with left lung expansion and with blood exchange between the heart and lungs. The heart may hit against the breastbone during exertion.
These uncomfortable, even painful, symptoms are unfortunately difficult to measure with current heart and lung function tests. This has made it difficult for many doctors (and, in the USA, insurance companies) to see PE as anything but a cosmetic problem. The symptoms are however very real and worrying for the PE patient, and are consistently described by all those who suffer them. They interfere greatly with leading a normal life as the child grows older. Doctors who acknowledge only the impact on self-image of living with such an obvious deformation may refuse to get involved in its correction because it's "only cosmetic". This is a great disservice to those with PE and their families.
As it progresses, PE can result in dramatically decreased exertion tolerance. Some notice that their stamina just isn't quite what it used to be. For others, even walking down the street becomes impossible without a rest stop. Any exertion may bring on chest discomfort and heart pounding, palpitations, cold sweats, shortness of breath, dizziness and great fatigue.
As the child with PE grows, his or her lowest ribs may develop an outward flaring shape. Sometimes this flaring is very pronounced -- and doctors may refer to this combination of protruding ribs plus breastbone depression as "mixed PE and PC". The flaring may happen because children with PE become strong diaphragm breathers to compensate for not getting good middle and upper chest inflation. This can produce a noticeable potbelly. Children with PE may be prone to chronic upper chest and bronchial complaints because they can�t effectively clear those areas of congestion.
Often the depression of PE seen in babies and young children is central, with the ribcage and chest still symmetrical in shape. Asymmetrical ribcage development becomes more noticeable (or appears for the first time) with the growth spurt of puberty. As the heart enlarges during the teens, it may be displaced leftward and twisted to fit into the compromised ribcage space. This can result in the left ribcage becoming larger than normal, while the right rib cage is relatively underdeveloped. If the right side develops less, the collarbone and upper ribs on that side may appear sunken, and the shoulder above may roll forward a bit. The sternum may become distorted as it is twisted up in response to the growth of the left side of the chest. For various reasons there may also be associated problems with the spine, most commonly scoliosis (a sideways S-curve) or loss of the thoracic curve ("straight back syndrome").
Unfortunately, the heart and lung symptoms experienced by so many with PE are hard to measure using present tests for cardio-pulmonary function. That does not make them less "real"! Among effects doctors do recognise are that pressure on the pulmonary artery (carrying blood from heart to lungs) may cause a heart murmur. The depressed breastbone can compress the heart itself, causing a decrease in ventricular volume or restriction of the aorta's root diameter, in proportion to the severity of the PE depression. This leads to mitral valve prolapse, common in PE as the patient gets older; and also mitral regurgitation (MR). Adult patients with severe PE especially can experience tachycardia, or abnormally fast heartbeat, worsening in response to exercise.
Surprisingly, few doctors admit that the heart "beating against" the depressed breastbone can cause discomfort! "Tests" can't measure it. Standard respiratory tests before corrective surgery are often just as inconclusive. Regardless of the problem with measurement, the common experience of patients who've undergone corrective surgery for PE is that they can afterwards exercise normally without breathing and circulatory discomfort. They report much improved breathing, whether or not they were aware of problems before the operation. Heart-related problems like MVP and abnormal rhythm often disappear once the breastbone is raised.
Yes! PE can be surgically corrected, with a high degree of success and patient satisfaction, including dramatic improvement to heart and lung function. Corrective surgery may be performed on patients of any age -- however, certain techniques are more used with patients in particular age groups, and some doctors limit their own practice to children.
Some of the medical literature suggests that there's an optimal age for PE correction, said to be between 8 and 10 years, and ideally before puberty. Correction before puberty may prevent the asymmetrical growth of the ribcage that can happen with PE during the teens. However, sometimes surgery on very young children is unavoidable due to the severity of the PE, which may be apparent at birth. Years ago the surgical technique occasionally interfered with the growth surfaces of the rib cage, impairing later chest development. Experienced PE surgeons today should be aware of this, and the techniques they use must not restrict future ribcage growth. Check out the PubMed references below, and always ASK for details of the procedure used by the surgeon you're considering.
There are three approaches commonly used to correct PE in the USA:
* the "traditional" approach -- a surgical reshaping of the ribcage and sternum, and insertion of an internal support device to help maintain the new shape during healing; the support device may or may not be removed later;
* the Leonard approach -- a surgical reshaping of the ribcage and sternum, with attachment of an external brace, anchored to the sternum, to be worn for 6 weeks or more during healing; the surgery is less extensive than the avove method;
* the Nuss approach -- insertion of a custom-shaped internal support bar across the ribcage beneath the depressed breastbone, then "flipping" it over to reverse the depression; this is left in for up to two to three years.
While the procedures pioneered by Drs. Nuss and Leonard are currently used only in the USA, variations on the traditional approach are available in other parts of the world.
In Brazil, Dr. Sydney Haje has developed a non-surgical method using an external brace and exercises to correct PC in children and adolescents. He has had some success in correcting PE with this approach too. And he is willing to advise doctors outside Brazil who are open to trying his technique with their patients. See below for Dr. Haje's contact details.
Follow the links on the Links to More Information page to learn what's involved with the Nuss Procedure, and with Dr. Leonard's approach (see Hal's story). In the following pages I'll focus on the traditional method of PE repair, which is what was used here in Australia to correct my 16-year-old daughter Gina's moderate-to-severe PE in July 1998.
Among US doctors using the traditional method is Dr. Eric Fonkalsrud, in California. He has produced a booklet describing PE and his corrective surgery, and he's very experienced and approachable. See the links to his web pages below -- and also the stories and photos on webpages by Kate and Ryan, and by Scott -- all of whom are Dr. Fonkalsrud's patients.
In the eastern US, Dr. Walter Pegoli at Johns Hopkins University Medical Center uses a similar procedure. And in Australia, Gina's surgeon, Mr. Keith Stokes at the Royal Children's Hospital in Melbourne, Victoria, also uses a very similar technique.
It is felt by many that the traditional approach gives the best correction for older teens and adults, especially when considerable asymmetry is involved. Dr. Fonkalsrud and others do use it effectively on younger children too. It is the most intrusive method, involving cutting and repositioning rib cartilages, the breastbone, and sometimes the rib bones. This method uses internal support, usually a thin surgical steel bar placed under the repositioned breastbone to hold it in its new position during healing. This is sutured or wired to a rib on either side, and can be left in permanently or removed 6 to 12 months later through a minor procedure.
The initial operation is long -- usually 2 to 4 hours, though Gina was there for 5 hours -- and the incision likewise is long, running across the chest under the breastline, or occasionally vertically up along the breastbone. If the surgeon uses subcutaneous stitching (which many seem to do now -- but ask!), the scar heals as a fine line. Because of the extensive nature of the surgery, the patient is in hospital from 4 to 7 days, and is usually on a narcotic drip or an epidural for pain control the first 3 days. The epidural in particular gives excellent relief and is in no way uncomfortable. Many young children find they require very little pain-killing medication after release from hospital. This seems amazing, given the major surgery.
There�s a long recovery period before the patient should be lifting, twisting, etc., varying according to how much bone reshaping was done. But by around 2 months after the operation, the ribcage should be pretty stable again, and by 6 to 9 months after the operation the healing should be complete. Most kids are back in school by the end of the first month post-op, though of course they must avoid any contact sports.