What is a pectus excavatum?Pectus excavatum is a Latin term meaning hollowed chest. Pectus excavatum can also be referred to as cobbler's chest, sunken chest, or funnel chest.
It is the most common congenital deformity of the anterior wall of the chest, in which several ribs and the sternum grow abnormally. This produces a caved-in or sunken appearance of the chest. It can either be present at birth or not develop until puberty.
Pectus excavatum occurs more often in men than women, appearing in 1 per every 300 to 400 white male births.
Approximately 40 percent of Pectus excavatum patients have one or more family members with the same defect.
Pectus excavatum is sometimes considered to be cosmetic; however, depending on the severity, it can impair cardiac and respiratory function and cause pain in the chest and back. People with the condition may experience negative psychosocial effects, and avoid activities that expose the chest.
What is the physiology of pectus excavatumHistorically, pectus excavatum was incorrectly considered as only a cosmetic defect. However, recent studies have revealed that children with significant defects also suffer from heart and breathing difficulties. The sunken chest restricts the volume of the chest and keeps the lung from expanding fully. Lung capacity may be reduced which can result in children have difficulty tolerating exercise or strenuous activity.
The sunken chest can also constrict the heart, reducing blood flood and heart function. An ultrasound of the chest (echocardiogram) often reveals some degree of compression and displacement of the heart. These effects on the heart and lung can be measured and are reversible by correcting the sunken chest. Mitral valve prolapse is found in 20-60% of patients evaluated for a pectus excavatum repair. It is important to note that these effects on the function of the heart and lung are not life threatening and that individuals with pectus excavatum can live a full and normal life even if the defect is not repaired.
How is pectus excavatum diagnosed?Before pectus excavatum can be properly treated, it must first be properly diagnosed. Your doctor and other specialists, including a pediatric surgeon will perform a complete physical exam and comprehensive blood tests to confirm the diagnosis.
Several other tests are required prior to the procedure.
- Pulmonary Function Test
- Computed tomography (CT) scan of the chest
- Echocardiogram (a picture of the heart)
- Allergy testing for nickel
An important measure that must be obtained prior to surgery is known as the “Haller Index.” Obtained by the CT of the chest, the Haller index is a mathematical measurement which defines the ratio of the transverse diameter (the horizontal distance of the inside of the ribcage) and the anteroposterior diameter (the shortest distance between the vertebrae and sternum. A normal Haller Index should be about 2.5. A Haller Index greater than 3.5 is considered severe and usually warrants surgical repair.
How is pectus excavatum surgically repaired at Yale?Surgical treatment done at Yale New Haven Children’s Hospital is called the “NUSS Procedure.”
Through two small incisions on either side of the chest, a curved steel bar (known as the Lorenz Pectus Bar) is inserted under the sternum. If your child has an allergy to nickel, the steel bar cannot be used. In this circumstance, we would make arrangements to order a custom bar made of titanium.
In addition, a separate, small incision is made to insert a tube with a camera to allow the surgeon to visualize the inside of the chest and insert tools in the remaining small incisions to complete the procedure.
Individually curved for each patient, the steel bar is used to ‘pop out’ the depression and is then fixed to the ribs on either side.
A small steel, grooved plate may be used at the end of the bar to help stabilize and attach the bar to the rib.
The bar is not visible from the outside and stays in place for a minimum of three years. When it is time, the bar is removed as an outpatient procedure.
What are the possible complications of the NUSS procedure?Possible complications from surgical repair of pectus excavatum include:
- Pneumothorax (an accumulation of air or gas in the pleural space)
- Pleural effusion (fluid around the lung)
- Infection: Will initially treat with antibiotics. If infection does not clear, bar may need to be removed
- Bar displacement
- Pectus excavatum recurrence (due to having the surgical correction completed too early prior to puberty and/or not leaving the strut or bar in place for a long enough period). Recommended age of repair is 14-16 years old.
What to expect during hospitalization
- Child will be admitted to the pediatric surgical unit
- Initial monitoring will be maintained to ensure the heart and lungs are okay
- Pain control will be very important. The pediatric pain service team will be involved in help coordinating all of your child’s pain medications including amount and frequency. They will also determine when medications can be weaned in preparation for discharge.
- Walking and deep breathing exercises are very important for post-operative recovery. The nursing team will help assist your child to ensure they are both safe and comfortable when ambulating.
- Plan to say in the hospital for approximately 5 days. Your child will be able to go home once eating, walking around, and pain is adequately controlled.
- Starting the second week after surgery, you may begin light activity, but still take it easy overall. Starting on the third week, you may resume most activities except ones specifically excluded by the surgeon.
- No karate, judo, gymnastics, or contact sports for the first three months.
- Avoid heavy lifting for the first two months.
- No backpacks for three months.
- Walking is excellent exercise and should be done frequently to build up your child’s strength. Your child should begin walking as soon as you arrive home.
- After the operation and for the first month at home, remind your child to bend at the hip, do not slouch or slump down when sitting. Good posture will help keep the bar in place.
- Your child will need to sleep on his/her back the first four weeks after surgery.
- Your child may bathe or swim 2 weeks after surgery
- Paper Band-Aids (Steri-Strips) on the incisions will slowly come off as your child bathes or showers. They can be completely removed after 7-10 days.
- CPR can be performed if it is needed. Compressions will need to be delivered with more force because of the steel bar.
- Defibrillation for cardiac arrest may be performed if needed. Front to back defibrillation pad placement is necessary while the bar is in place.
- You will be given an application for a medical alert bracelet at discharge. The bracelet will alert Emergency Medical Services personnel and other healthcare providers about the steel bar. This will let them know to push harder during compressions and to place defibrillation pads correctly.
- If your child is scheduled for a Magnetic Resonance Imaging (MRI) exam in the future, inform the radiologist that your child has a pectus bar. An MRI can usually be done with the bar in place if the radiologist adjusts the MRI equipment settings.
Call the doctor if any of these problems develop
- Fever (temperature greater than 100.5 degrees Fahrenheit)
- Persistent coughing (does not stop)
- Chest pain, especially with deep breaths
- Redness, drainage or swelling at the incision sites under the arms
- Any breathing difficulties
- Any injury to the chest that could have caused the bar to move