What is pectus excavatum?
Pectus excavatum (PE) is an abnormal development of the rib cage where the breastbone (sternum) caves in, resulting in a sunken chest wall deformity. Sometimes referred to as “funnel chest,” pectus excavatum is a deformity often present at birth (congenital) that can be mild or severe.
What causes pectus excavatum?
The cause of pectus excavatum is not well understood. Yet, researchers believe that the deformity is caused by excessive growth of the connective tissue (cartilage) that joins the ribs to the breastbone (also known as the costochondral region), which causes an inward defect of the sternum.
While the vast majority of pectus excavatum cases are not associated with any other condition, some disorders may include the sunken chest feature of pectus excavatum, including:
Marfan syndrome: A connective tissue disorder, which causes skeletal defects typically recognized by long limbs and ‘spider-like’ fingers, chest abnormalities, curvature of the spine and certain facial features including a highly arched palate, and crowded teeth.
Rickets: A deficiency disease occurring primarily in children, Rickets results from a lack of vitamin D or calcium and from insufficient exposure to sunlight, which disturbs normal bone growth.
Scoliosis: A curvature of the spine.
What are the symptoms of pectus excavatum?
Most patients do not have symptoms, though a minority of patients may have the following symptoms:
- Fatigue
- Shortness of breath
- Chest pain
- Fast heart rate (tachycardia)
Who is affected by pectus excavatum?
Pectus excavatum is a fairly common congenital deformity that accounts for approximately 90 percent of congenital wall deformities. Approximately 40 percent of pectus excavatum patients have one or more family members with the same defect.
Pectus excavatum occurs more often in men than women, appearing in 1 per every 300 to 400 white male births.
Is pectus excavatum serious?
Pectus excavatum is not preventable, but the condition is treatable. In rare cases if the condition is left untreated, pectus excavatum can cause compression of the heart and lungs.
In addition to pectus excavatum causing an unattractive appearance of the chest wall, the condition can also force the heart into the left chest to varying degrees, depending on the severity of the defect.
Contact your doctor if the following symptoms occur:
- Trouble breathing
- Decreased exercise tolerance
- Chest pain
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 thoracic surgeon and pulmonologist, will perform a complete physical exam and comprehensive blood tests to confirm the diagnosis.
Several other tests may be performed, including:
- Physical (stress) test
- Pulmonary function test
- Laboratory studies (blood work), such as chromosome studies or enzyme assays
- Metabolic studies
- Chest x-ray
- Computed tomography (CT) scan of the chest
- Electrocardiogram (EKG)
- Echocardiogram (a picture of the heart)
How is pectus excavatum treated?
Since most patients with the deformity do not have symptoms, treatment may not be needed, or will be dependent upon the development of symptoms.
Physical therapy in young patients (under the age of 18, due to most pectus deformities remaining the same after this age) may play a role in slowing the development of the chest wall deformity and may possibly reverse some of the chest wall deformity.
If pectus excavatum is compromising either the heart or lungs, your doctor may recommend surgery. A recent review of 169 surgeries performed to correct pectus excavatum between January 1960 and May 2005 showed that surgical repair significantly improves heart and blood vessel (cardiovascular) function.
Surgery: The primary goal of pectus excavatum repair surgery is to correct the chest deformity to improve a patient’s breathing, posture and cardiac function. This is typically accomplished by removing a portion of the deformed cartilage and repositioning the breastbone.
A variety of surgical procedures are available to repair pectus excavatum, including:
Modified Ravitch technique: Originally completed by a long incision across the chest to resect excess cartilage, reposition rib bones, and implant a wedge bone graft to correct pectus excavatum, the Ravitch technique has been recently modified as a less-invasive procedure.
The modified Ravitch technique is completed with a vertical incision in the mid-chest area to remove anterior cartilage. A horizontal cut is then made in the sternum to allow it to bend forward. In the original procedure one or two stainless-steel struts are placed across the anterior chest and anchored to the ribs laterally to support the breastbone in the new anterior position. The struts are not visible from the outside and are removed after two years during a surgical procedure. More recently, surgeons have been using permanent titanium plates to stabilize the sternum in adults and teens who have completed their growth. These plates need not be removed and may prevent recurrence of the defect that can be seen following strut removal. There appears to be less post-operative pain with plate use although this claim awaits more rigorous testing.
The Nuss Procedure: Usually restricted for adolescent patients, surgeons use a video-assisted thoracoscopic surgery (VATS) technique to correct pectus excavatum.
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. Individually curved for each patient, the steel bar is used to correct 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 two years. When it is time, the bar is removed in an outpatient procedure visit.
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.Your surgeon will determine the best surgical approach to correct your condition.
What are the benefits of pectus excavatum surgical repair?
The goal of pectus excavatum repair is to alleviate compression of the heart and lungs that may impair function. The expectation is that patients will have improved exercise tolerance and endurance with appropriate physical therapy to enhance their recovery.
What are the risks of surgical repair of pectus excavatum?
The surgical repair of pectus excavatum, like other extensive surgical procedures, presents risks. While both the Nuss procedure and the modified Ravitch technique are safe and effective procedures, complications can occur.
Possible complications from surgical repair of pectus excavatum include:
- Pneumothorax (an accumulation of air or gas in the pleural space)
- Bleeding
- Pleural effusion (fluid around the lung)
- Infection
- Bar displacement or erosion through the skin
- 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). Recurrence is less likely after the Ravitch procedure.
