What is pectus excavatum

Pectus excavatum is a congenital chest wall deformity that is caused by growth abnormality of the cartilage that connects the ribs to the breastbone (sternum). This causes a depression of the sternum and the chest has a “sunken in” or “funnel chest” appearance. The condition affects more boys than girls. It is often present at birth but becomes more noticeable during times of rapid growth, such as puberty.

Symptoms

Pectus excavatum can range from mild to severe. Those with a mild condition often do not have symptoms. Those with moderate-to-severe condition may have symptoms such as shortness of breath, fatigue, exercise intolerance/limitations and chest pain. With a severe case of pectus excavatum, there can be compression of the heart. Pectus excavatum may also be associated with scoliosis in some children. Children with pectus excavatum may experience some negative effects on their self-esteem due to the appearance of the chest.

Causes

There is no known cause for pectus excavatum. It can sometimes run in families — which suggests genetics may play a role. Pectus excavatum can also be associated with connective tissue disorders such as Marfan syndrome.

Diagnosis

Pectus excavatum is diagnosed by a thorough health history and physical examination. Diagnostic imaging tests are used to get a detailed look at your child’s condition. 

A CT scan of the chest is essential as it provides the Haller index. The Haller index is the ratio between the transverse chest diameter (measurement from one side of the ribcage to the other) and the anteroposterior diameter (measurement from the breastbone to the spinal cord) of the chest.

A normal chest ratio is approximately 2.5 cm and an index over 3.2 cm is often defined as severe and eligible for the Nuss procedure. The chest CT will also show any displacement or compression of the heart.

For children and adolescents, it’s especially important to have imaging tests done at an institution with pediatric radiologists who specialize in low-dose imaging techniques that reduce patients’ exposure to radiation. CHOP is a long-time leader in the movement toward safer scanning for children.

Some other tests such as pulmonary function tests (PFT) or an echocardiogram may be used to determine the extent of heart and lung compression. Your insurance company may require these tests before approving surgery.

Treatment of pectus excavatum

The treatment of pectus excavatum is dependent upon the severity of the defect and your child’s symptoms. In most cases, surgery is not indicated. At CHOP, we offer two different treatments for pectus excavatum. We may recommend observation for mild to moderate cases and surgery for more severe or complex cases. Early referral to a pediatric surgeon is important to determine surgical options and optimize treatment.

• The Nuss procedure is a minimally invasive technique for surgical repair that involves placement of a concave steel bar under the sternum. At CHOP, the Nuss procedure is the most common surgical procedure for pectus excavatum. Our experienced pediatric surgeons have performed more than 500 Nuss procedures, and typically perform approximately 40 cases each year. Learn what to expect during pectus excavatum surgery »
• If your child is a surgical candidate and has twisting (torsion) of the chest, the Ravitch procedure may be a better option.

Although surgery may be recommended for your child, the decision to move forward with the procedure is up to your child and family.

Depending on your insurance, you may need a prior authorization prior to your clinic visits. When you schedule your appointment, you will be notified if you need a prior authorization/referral. Your primary care physician can help you obtain a referral/prior authorization for your clinic visit. If surgery is recommended for your child, a member of our team will assist you in obtaining any authorizations needed.

In addition, you will need to obtain allergy testing to ensure your child does not have a sensitivity to nickel or chromium, tiny components present within the stainless steel bar. If your child tests positive to nickel or chromium, they will need a custom-made titanium bar.

Follow-up care

After surgery, your child will stay in the hospital for a few days to recover. The length of stay will depend largely on pain management. Our dedicated Pain Management Program team will help make sure your child's pain is controlled. They will also begin physical therapy, which is important to promote recovery and movement, regain strength, expand the lungs, promote bowel function, and prevent possible complications.

After being discharged from the hospital, your child will follow up in the general surgery clinic in 2-4 weeks. Your child’s surgeon or nurse practitioner will go over pain management and activity restrictions, and any other information you may need to care for your child at home. For more information about what to expect after surgery, read more about the Nuss procedure.

While it is not mandatory to wear a Medical Alert bracelet after surgical correction of pectus excavatum, it is strongly recommended. Information can be provided to you at a post-operative visit on obtaining a Medical Alert bracelet. The inscription on the bracelet should read “steel bar in chest, CPR more force, cardioversion ant/post placement.”

Having a steel bar in the chest raises several common questions about daily activities. Here are a few things you should know:

• The bar should not set off metal detectors in the airport, but depending on the type of material, it may in some cases (titanium bar is more likely to set off detectors, while stainless steel will not). We can provide documentation of the bar if necessary.
• Antibiotics are not required prior to dental procedures while the bar is in place.
• If needed, your child can still receive CPR while the bar is in place. Chest compressions should be done with more force due to the bar placement. An AED can also be used with the bar in place; however, the paddle placement will be different. One paddle should be placed on the front of the chest and the other should be on the back.