What is pectus excavatum and its symptoms?
Pectus excavatum is caused by the abnormal growth in the chest of the connective tissues (cartilage) that attach the breastbone (sternum) to the ribs. This causes a depression in the chest that can range from mild to severe. Both or just one side of the breastbone may be affected. While most patients do not have symptoms, those with severe forms of the abnormality may experience:
- Shortness of breath
- Chest pain
- Older children may develop issues involving self-image or self-confidence
What causes pectus excavatum?
The cause of the disorder is not known. However, it tends to occur in families, which may mean that genetics play a role. About 40 percent of children diagnosed with the disorder have one or more family members with the same defect. The condition also can be associated with inherited connective tissue disorders like Marfan syndrome and Ehlers-Danlos syndrome, as well as with rickets and scoliosis.
How is pectus excavatum diagnosed?
Most cases of pectus excavatum are identified when children are still infants. The condition can become more noticeable during puberty, when rapid bone and cartilage growth occurs.
St. Louis Children’s Hospital pediatric surgeons recommend that children undergo evaluation as soon as the possibility of pectus excavatum is suspected. The early diagnosis allows physicians to follow patients as they grow and ensure they don’t develop symptoms, or that existing symptoms do not worsen.
Diagnosing pectus excavatum involves:
- A thorough physical exam
- Detailed family history
- X-rays to help determine the severity of the depression
- If necessary, diagnostic imaging scans like computed tomography (CT) or magnetic resonance imaging (MRI) to define the chest’s anatomy
- Echocardiogram and electrocardiogram to test heart function
- Pulmonary function tests to detect breathing problems
- Genetic testing for those who are suspected of having related syndromes
How is pectus excavatum treated?
To ensure the best result for children with pectus excavatum, the correct timing of treatment is important. Intervening too early can create problems because the possibility exists that future growth spurts could affect patients’ chest development and make treatment ineffective. Waiting too long—for instance, until patients are in their late teens—can make repairs more difficult because the chest wall is less compliant.
For children without symptoms and therefore not in need of immediate intervention, the optimum time for treatment is when they reach puberty—usually the early teens. Two surgical options are available:
- Ravitch technique—Requires an incision across the sternum, through which the surgeon removes the abnormally growing cartilage attached to the ribs. The sternum is then set over a stainless steel bar that is secured into position and remains in place until the cartilage regrows, usually within six to 10 months. The bar is then removed surgically.
- Nuss technique—Uses two small incisions on either side of the chest under the arms. A curved steel bar designed to apply pressure and remodel the cartilage—rather than removing it—is inserted under the sternum. The remodeling process requires that the bar remain in place for two to three years before it may be removed.
Although there are instances when one of these procedures is better suited to a patient’s condition, many times the decision about the type of surgery is made by patients and their families. Factors influencing that decision include:
- The length of time patients are restricted from participating in various sports such as wrestling, baseball and golf while a bar is implanted; the Ravitch requires less time with a bar in place.
- The importance of cosmetic value; the Nuss has smaller, less visible incisions.
Both procedures require a four- to five-day hospital stay following surgery, and pain medication for several weeks to manage discomfort. The risks for the surgery are equivalent and the success rates similar.
Some patients with pectus excavatum may decide not to have surgery because their condition is mild and they are not bothered by their appearance. In addition, some young athletes may forego surgery once testing shows their exercise tolerance and performance are not affected by their condition.
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